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AnemiaAnemia

Classification of anemiaClassification of anemia

HypoproliferativeHypoproliferative

--Iron deficiency anemiaIron deficiency anemia

--Vit. B12 deficiency anemia (megaloblastic)Vit. B12 deficiency anemia (megaloblastic)

--Folate deficiency (megaloblastic)Folate deficiency (megaloblastic)

--Decreased erythropoietin productionDecreased erythropoietin production

--Cancer/InflammationCancer/Inflammation

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Hemolytic AnemiaHemolytic Anemia

Sickle Cell AnemiaSickle Cell AnemiaThalassemiaThalassemia

Immune Hemolytic anemiaImmune Hemolytic anemia

Hereditary HemochromatosisHereditary Hemochromatosis

PolycythemiaPolycythemia

LeukopeniaLeukopenia

LeukemiaLeukemia

Agnogenic Myeloid MetaplasiaAgnogenic Myeloid MetaplasiaLymphomaLymphoma

Multiple MyolomaMultiple Myoloma

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Bleeding AnemiaBleeding Anemia

Primary ThrombocythemiaPrimary ThrombocythemiaSecondary ThrombocythemiaSecondary Thrombocythemia

thrombocythemiathrombocythemia

Idiopathic Thrombocytopenic PurpuraIdiopathic Thrombocytopenic Purpura

Platelet DefectsPlatelet Defects

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Hypoproliferative AnemiaHypoproliferative Anemia

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Iron deficiency AnemiaIron deficiency Anemia

is a condition where a person has inadequateis a condition where a person has inadequate

amounts of iron to meet body demands. It is aamounts of iron to meet body demands. It is a

decrease in the amount of red cells in the blooddecrease in the amount of red cells in the bloodcaused by having too little iron. Ironcaused by having too little iron. Iron

deficiency anemia is usually caused by adeficiency anemia is usually caused by a dietdiet

insufficient in iron or from blood loss. Bloodinsufficient in iron or from blood loss. Blood

loss can be acute as in hemorrhage or traumaloss can be acute as in hemorrhage or traumaor long term as in heavy menstruation.or long term as in heavy menstruation.

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SymptomsSymptoms

In general, anemia causes extreme fatigue, pale skin, weakness,In general, anemia causes extreme fatigue, pale skin, weakness,shortness of breath, headache, lightheadedness, and often coldshortness of breath, headache, lightheadedness, and often coldhands and feet.hands and feet.Iron deficiency anemia symptoms also may include:Iron deficiency anemia symptoms also may include:

Inflammation or soreness of your tongueInflammation or soreness of your tongue

Brittle nailsBrittle nails

Unusual cravings for nonUnusual cravings for non--nutritive substances, such as ice, dirt ornutritive substances, such as ice, dirt orpure starchpure starch

Poor appetite, especially in infants and children with iron deficiencyPoor appetite, especially in infants and children with iron deficiencyanemiaanemia

Some people with iron deficiency anemia experience restless legsSome people with iron deficiency anemia experience restless legssyndromesyndrome an uncomfortable tingling or crawling feeling in youran uncomfortable tingling or crawling feeling in yourlegs that's generally relieved by moving them.legs that's generally relieved by moving them.If you or your child develops signs and symptoms that suggest ironIf you or your child develops signs and symptoms that suggest irondeficiency anemia, see your doctor. Initially, iron deficiency anemiadeficiency anemia, see your doctor. Initially, iron deficiency anemiacan be so mild that it goes unnoticed. But as the body becomescan be so mild that it goes unnoticed. But as the body becomesmore deficient in iron and anemia worsens, the signs and symptomsmore deficient in iron and anemia worsens, the signs and symptoms

intensifyintensify

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TreatmentsTreatments

Increased intake of ironIncreased intake of iron--rich foods is beneficial, but usually isn'trich foods is beneficial, but usually isn'tenough to correct the problem. You need iron supplementationenough to correct the problem. You need iron supplementationto build back your iron reserves, as well as to meet your body'sto build back your iron reserves, as well as to meet your body'sdaily iron requirements. In pregnant women, iron supplementsdaily iron requirements. In pregnant women, iron supplementshelp provide enough iron for both the mother and her fetus.help provide enough iron for both the mother and her fetus.

For children or adults with mild iron deficiency anemiaFor children or adults with mild iron deficiency anemia-- doctorsdoctors

may recommend a daily multivitamin containing iron. Butmay recommend a daily multivitamin containing iron. Buttypically, doctors recommend iron tabletstypically, doctors recommend iron tablets such assuch asprescription ferrous sulfate tablets or an overprescription ferrous sulfate tablets or an over--thethe--countercountersupplement. These oral iron supplements are usually bestsupplement. These oral iron supplements are usually bestabsorbed in an empty stomach. However, because iron canabsorbed in an empty stomach. However, because iron canirritate your stomach, you may need to take the supplementsirritate your stomach, you may need to take the supplementswith food. Your doctor may recommend that you take ironwith food. Your doctor may recommend that you take ironsupplements with orange juice or with a vitamin C tablet.supplements with orange juice or with a vitamin C tablet.Vitamin C, in orange juice or tablet form, helps increase ironVitamin C, in orange juice or tablet form, helps increase ironabsorption.absorption.

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Anemia of Renal DiseaseAnemia of Renal Disease

Anemia of renal disease occurs in both acute and chronic renalAnemia of renal disease occurs in both acute and chronic renalfailure. Renal excretory dysfunction and endocrinefailure. Renal excretory dysfunction and endocrineabnormalities play pathophysiologic roles. This isabnormalities play pathophysiologic roles. This isdemonstrated by the builddemonstrated by the build--up of uremic toxins and decreasedup of uremic toxins and decreasederythropoietin production, both of which adversely affecterythropoietin production, both of which adversely affecterythropoiesis. The accumulation of toxic metabolites, whicherythropoiesis. The accumulation of toxic metabolites, whichare normally excreted by the kidneys, shortens the life span ofare normally excreted by the kidneys, shortens the life span ofcirculating red blood cells. In fact, there is an inversecirculating red blood cells. In fact, there is an inverserelationship between blood urea nitrogen (BUN) levels and redrelationship between blood urea nitrogen (BUN) levels and red

blood cell life span. More important, however, is theblood cell life span. More important, however, is the

diminished renal production or the dysfunction ofdiminished renal production or the dysfunction oferythropoietin that results in decreased red blood cellerythropoietin that results in decreased red blood cellproduction. Although the erythropoietin levels may be low,production. Although the erythropoietin levels may be low,normal or high, they are inappropriately low when adjusted fornormal or high, they are inappropriately low when adjusted forthe degree of anemia.the degree of anemia.

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The anemia of renal disease is usually normocytic and normochromic, butThe anemia of renal disease is usually normocytic and normochromic, butmicrocytosis can also occur. Hemoglobin concentrations may be mildlymicrocytosis can also occur. Hemoglobin concentrations may be mildlydecreased with lesser degrees of azotemia or markedly diminished indecreased with lesser degrees of azotemia or markedly diminished in

chronic renal failure (hemoglobin levels as low as 5 to 7 g per dL [50 to 70chronic renal failure (hemoglobin levels as low as 5 to 7 g per dL [50 to 70g per L]). A variety of morphologic abnormalities can be seen, such as burrg per L]). A variety of morphologic abnormalities can be seen, such as burrcells in cases of uremia; microcytic, hypochromic red blood cells despitecells in cases of uremia; microcytic, hypochromic red blood cells despitenormal iron stores; or schistocytes and thrombocytopenia in cases ofnormal iron stores; or schistocytes and thrombocytopenia in cases ofdisseminated intravascular coagulation or thrombotic thrombocytopenicdisseminated intravascular coagulation or thrombotic thrombocytopenicpurpura.purpura.

Androgens were used in the past in an attempt to stimulate red blood cellAndrogens were used in the past in an attempt to stimulate red blood cellproduction, but the administration of erythropoietin has effectively replacedproduction, but the administration of erythropoietin has effectively replacedthis treatment. In patients with a relative deficiency of erythropoietin, boththis treatment. In patients with a relative deficiency of erythropoietin, bothintravenous and subcutaneous administration of erythropoietin increasesintravenous and subcutaneous administration of erythropoietin increasesthe reticulocyte count and red blood cell mass in a dosethe reticulocyte count and red blood cell mass in a dose--related manner.3related manner.3Even when iron stores are adequate, iron supplementation is usually neededEven when iron stores are adequate, iron supplementation is usually neededwith erythropoietin administration because enhanced red blood cellwith erythropoietin administration because enhanced red blood cell

production rapidly depletes the stores. Erythropoietin can be given threeproduction rapidly depletes the stores. Erythropoietin can be given threetimes per week, but higher dosages on a oncetimes per week, but higher dosages on a once--perper--week basis may also beweek basis may also beeffectiveeffective

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Anemia of Chronic DiseaseAnemia of Chronic Disease Anemia of chronic disease is a blood disorder that results from a medicalAnemia of chronic disease is a blood disorder that results from a medical

condition that affects the production and lifespan of red blood cells.condition that affects the production and lifespan of red blood cells.

Increasingly referred to as "anemia of inflammation", is a form ofIncreasingly referred to as "anemia of inflammation", is a form ofanemiaanemiaseen in chronic illness, e.g. from chronic infection, chronic immuneseen in chronic illness, e.g. from chronic infection, chronic immuneactivation, or malignancy.activation, or malignancy.

The precise pathophysiologic mechanism of ACD is unclear. The anemia isThe precise pathophysiologic mechanism of ACD is unclear. The anemia is

related more to decreased red blood cell production than to increasedrelated more to decreased red blood cell production than to increaseddestruction. There is an impair ment in the transport of iron from irondestruction. There is an impair ment in the transport of iron from ironstorage sites, such as the liver and bone marrow, to red blood cells (Figurestorage sites, such as the liver and bone marrow, to red blood cells (Figure2). Infection or inflammation may be responsible for the production of2). Infection or inflammation may be responsible for the production ofmediators (such as interleukinmediators (such as interleukin--1) that result in increased sequestration of1) that result in increased sequestration ofiron in macrophages and hepatocytes. Additional factors are decreasediron in macrophages and hepatocytes. Additional factors are decreasedlevels of transferrin (TIBC) and competition with another transport proteinlevels of transferrin (TIBC) and competition with another transport protein

(lactoferrin) that preferentially returns iron back to macrophages (Figure 2).(lactoferrin) that preferentially returns iron back to macrophages (Figure 2).Therefore, iron is present but inaccessible for use in the production ofTherefore, iron is present but inaccessible for use in the production ofhemoglobin within erythrocytes. A poor response to or a relative decreasehemoglobin within erythrocytes. A poor response to or a relative decreasein erythropoietin production complicates the anemia.4 Finally, anin erythropoietin production complicates the anemia.4 Finally, anunexplained slightly shortened red blood cell survival also contributes tounexplained slightly shortened red blood cell survival also contributes tothe anemia.the anemia.

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..Diagram of iron kinetics from iron stores toDiagram of iron kinetics from iron stores todeveloping red blood cell (RBC). Iron storesdeveloping red blood cell (RBC). Iron stores

include the bone marrow, reticuloendothelialinclude the bone marrow, reticuloendothelialsystem (liver and spleen) and RBCs.system (liver and spleen) and RBCs.Transferrin (total ironTransferrin (total iron--binding capacity [TIBC])binding capacity [TIBC])transports iron (Fe) to developing erythrocytes.transports iron (Fe) to developing erythrocytes.Iron is deposited in the RBC, and transferrinIron is deposited in the RBC, and transferrinreturns to storage sites to bind more Fe forreturns to storage sites to bind more Fe fortransport. Lactoferrin is a competitor oftransport. Lactoferrin is a competitor of

transferrin; it takes Fe that is free and returns ittransferrin; it takes Fe that is free and returns itto storage sites. Lactoferrin levels are elevatedto storage sites. Lactoferrin levels are elevatedin anemia of chronic disease. Increases inin anemia of chronic disease. Increases ininterleukininterleukin--1 increase the sequestration of Fe1 increase the sequestration of Fe

in storage sites. (Hb=hemoglobin)in storage sites. (Hb=hemoglobin)

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Sections of peripheral blood smears from twoSections of peripheral blood smears from two

patients with anemia of chronic disease.patients with anemia of chronic disease. (Left)(Left)

Normal lymphocyte; normal red blood cellNormal lymphocyte; normal red blood cell

sizes approximate the diameter of a maturesizes approximate the diameter of a mature

lymphocyte. The erythrocytes shown here arelymphocyte. The erythrocytes shown here are

normal to mildly microcytic.normal to mildly microcytic. (Right)(Right) RedRedblood cells here are also slightly microcytic;blood cells here are also slightly microcytic;

however, the polymorphonuclear leukocytehowever, the polymorphonuclear leukocyte

contains excessive granules (toxic granulation),contains excessive granules (toxic granulation),which suggests an underlying infection orwhich suggests an underlying infection or

inflammation. (Oil immersion;inflammation. (Oil immersion; 1,000)1,000)

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TreatmentTreatment

The ideal treatment for anemia of chronic disease is to treat the chronic diseaseThe ideal treatment for anemia of chronic disease is to treat the chronic diseasesuccessfully. Barring that, many patients with anemia of chronic diseasesuccessfully. Barring that, many patients with anemia of chronic diseasesimply live with the effects of the anemia as part of enduring the limitssimply live with the effects of the anemia as part of enduring the limitsplaced on them by other aspects of their underlying medical conditions. Inplaced on them by other aspects of their underlying medical conditions. Inmore severe cases,more severe cases, transfusionstransfusions or several versions of commerciallyor several versions of commercially--producedproduced erythropoietinerythropoietin can be helpful in some circumstances; bothcan be helpful in some circumstances; bothapproaches are costlyapproaches are costly

The treatment of ACD is directed at the underlying cause. Iron therapy is of noThe treatment of ACD is directed at the underlying cause. Iron therapy is of nobenefit. If necessary, transfusions or erythropoietin therapy can bebenefit. If necessary, transfusions or erythropoietin therapy can beconsidered when the degree of anemia poses cardiovascular problems.considered when the degree of anemia poses cardiovascular problems.When ACD is associated with cancer, other factors may contribute to theWhen ACD is associated with cancer, other factors may contribute to the

anemia, such as transient bone marrow suppression from chemotherapy oranemia, such as transient bone marrow suppression from chemotherapy orbone marrow replacement by tumor. In patients with cancer, treatment withbone marrow replacement by tumor. In patients with cancer, treatment withtransfusions or erythropoietin is acceptable because of greater degrees oftransfusions or erythropoietin is acceptable because of greater degrees ofanemia and other complicating features such as bleeding,anemia and other complicating features such as bleeding,thrombocytopenia and relative deficiency of erythropoietinthrombocytopenia and relative deficiency of erythropoietin

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Aplastic AnemiaAplastic Anemia

is a condition whereis a condition wherebone marrowbone marrow does not producedoes not produce

sufficient newsufficient new cellscells to replenishto replenishblood cellsblood cells..

The term 'aplastic' means the marrow suffers from anThe term 'aplastic' means the marrow suffers from an

aplasiaaplasia that renders it unable to function properly.that renders it unable to function properly.

AnemiaAnemia is the condition of having reducedis the condition of having reduced

hemoglobinhemoglobin or red cell concentration in the blood.or red cell concentration in the blood.

Typically, anemia refers to low red blood cell counts,Typically, anemia refers to low red blood cell counts,

but aplastic anemia patients have lower counts of allbut aplastic anemia patients have lower counts of all

three blood cell types:three blood cell types: red blood cellsred blood cells,, white bloodwhite blood

cellscells, and, andplateletsplatelets, termed, termedpancytopeniapancytopenia..

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Signs and symptomsSigns and symptoms

AnemiaAnemia withwith malaisemalaise,,pallorpallorand associatedand associatedsymptomssymptoms

ThrombocytopeniaThrombocytopenia (low platelet counts),(low platelet counts),

leading to increased risk ofleading to increased risk ofhemorrhagehemorrhage andandbruisingbruising

LeukopeniaLeukopenia (low white blood cell count),(low white blood cell count),

leading to increased risk ofleading to increased risk ofinfectioninfection

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Patient with aplastic anemiaPatient with aplastic anemia

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Clinical ManifestationsClinical Manifestations

The manifstation of aplastic anemia are often insidious.The manifstation of aplastic anemia are often insidious.

Complications resulting from bone marrow failure mayComplications resulting from bone marrow failure mayoccur before the diagnosis is established. Typicaloccur before the diagnosis is established. Typical

complications are infection and symptoms of anemiacomplications are infection and symptoms of anemia(fatigue, pallor, dyspnea). Purpura (bruising) may(fatigue, pallor, dyspnea). Purpura (bruising) maydevelop after and should trigger a CBC anddevelop after and should trigger a CBC andhematologic evaluation if these were not performedhematologic evaluation if these were not performedinitially. If the patient has had repeated throatinitially. If the patient has had repeated throatinfections, cervical lymphadenopathy may be seen.infections, cervical lymphadenopathy may be seen.Other lymphodenopathies and spleomegalyOther lymphodenopathies and spleomegalysometimes occur. Retinal hemorrhages are common.sometimes occur. Retinal hemorrhages are common.

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Megaloblastic AnemiaMegaloblastic Anemia

anemiaanemia is anis an anemiaanemia (of(ofmacrocyticmacrocytic classification)classification)which results from inhibition of DNA synthesis inwhich results from inhibition of DNA synthesis inred blood cell production. It is often due tored blood cell production. It is often due todeficiency ofdeficiency ofvitamin B12vitamin B12 and/orand/orfolic acidfolic acid. It can. It can

be the result of a lack ofbe the result of a lack ofintrinsic factorintrinsic factor(which lack(which lackinterferes with B12 absorption), causinginterferes with B12 absorption), causingperniciousperniciousanemiaanemia, or with other antimetabolites which poison, or with other antimetabolites which poisonDNA production, such as chemotherapeutic agents.DNA production, such as chemotherapeutic agents.

It is characterized by many large immature andIt is characterized by many large immature anddysfunctional red blood cells (dysfunctional red blood cells (megaloblastsmegaloblasts) in the) in thebone marrow,bone marrow,[1][1] and also byand also by hypersegmentedhypersegmented orormultisegmentedmultisegmented neutrophilsneutrophils..

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Signs and symptoms:Signs and symptoms:

Loss of appetiteLoss of appetite DiarrheaDiarrhea

Tingling and numbness of hand and feetTingling and numbness of hand and feet

Pale skin colorPale skin color TirednessTiredness

HeadachesHeadaches

Sore mouth and tongueSore mouth and tongue Change in skin colorChange in skin color

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Patient with Megaloblastic AnemiaPatient with Megaloblastic Anemia

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Hemolytic AnemiaHemolytic Anemia

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Sickle Cell AnemiaSickle Cell Anemia

is ais ablood disorderblood disordercharacterized bycharacterized by red blood cellsred blood cells that assume anthat assume anabnormal, rigid,abnormal, rigid, sicklesickle shape. Sickling decreases the cells' flexibility andshape. Sickling decreases the cells' flexibility andresults in their restricted movement throughresults in their restricted movement throughblood vesselsblood vessels, depriving, deprivingdownstream tissues ofdownstream tissues ofoxygenoxygen. The disease is chronic and lifelong:. The disease is chronic and lifelong:individuals are most often well, but their lives are punctuated by periodicindividuals are most often well, but their lives are punctuated by periodicpainful attacks and a risk of various other complications. Life expectancy ispainful attacks and a risk of various other complications. Life expectancy isshortened, with older studies reporting an average life expectancy of 42 andshortened, with older studies reporting an average life expectancy of 42 and48 years for males and females, respectively.48 years for males and females, respectively.[1][1]

SickleSickle--cell disease occurs more commonly in people (or their descendants)cell disease occurs more commonly in people (or their descendants)from parts of the world such asfrom parts of the world such as subsub--Saharan AfricaSaharan Africa, where, where malariamalaria is oris orwas common, but it also occurs in people of other ethnicities. This iswas common, but it also occurs in people of other ethnicities. This is

because those with one or twobecause those with one or two allelesalleles of the sickleof the sickle--cell disease are resistantcell disease are resistantto malaria since the sickle red blood cells are not conducive to the parasitesto malaria since the sickle red blood cells are not conducive to the parasites-- in areas where malaria is common there is ain areas where malaria is common there is a survival valuesurvival value in carrying thein carrying thesicklesickle--cell genes.cell genes.

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Signs and SymptomsSigns and Symptoms

Chronic hemolysis or thrombosisChronic hemolysis or thrombosis Hemoglobin values of 7Hemoglobin values of 7--10g/dl10g/dl

JaundiceJaundice

Bone marrow expandBone marrow expand TachycardiaTachycardia

Cardiac murmursCardiac murmurs

DysrythmiasDysrythmias Heart failureHeart failure

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Patient with Sickle cell anemiaPatient with Sickle cell anemia

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Nursing InterventionNursing Intervention

Avoid situations that increase cellular metabolism; strenuousAvoid situations that increase cellular metabolism; strenuousphysical activity, contact sports, emotional stress, highphysical activity, contact sports, emotional stress, highaltitudes, known sources of infectionaltitudes, known sources of infection

Report fever or mild infection at once to parents for them toReport fever or mild infection at once to parents for them toseek medical attention immediatelyseek medical attention immediately

3) Encourage fluid intake to prevent sickling and blood3) Encourage fluid intake to prevent sickling and bloodstasisstasis

# of glasses of water or fluids per day/ # in the school setting# of glasses of water or fluids per day/ # in the school setting 4.)Maintain up to date immunization4.)Maintain up to date immunization

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ThalassemiaThalassemia

is an inheritedis an inherited autosomal recessiveautosomal recessive bloodblood

diseasedisease. In thalassemia, the genetic defect. In thalassemia, the genetic defect

results in reduced rate of synthesis of one ofresults in reduced rate of synthesis of one of

the globin chains that make upthe globin chains that make up hemoglobinhemoglobin..

Reduced synthesis of one of the globin chainsReduced synthesis of one of the globin chains

can cause the formation of abnormalcan cause the formation of abnormal

haemoglobin molecules, and this in turn causeshaemoglobin molecules, and this in turn causesthethe anemiaanemia which is the characteristicwhich is the characteristic

presenting symptom of the Thalassemia.presenting symptom of the Thalassemia.

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Weakness.Weakness.

Fatigue.Fatigue.

Lightheadedness.Lightheadedness.

Skin that looks paler than normal.Skin that looks paler than normal.

Jaundice (skin and whites of the eyes appear yellow).Jaundice (skin and whites of the eyes appear yellow).

Dark urine.Dark urine. Decreased appetite and weight loss (poor growth in a child).Decreased appetite and weight loss (poor growth in a child).

A rapid heartbeat.A rapid heartbeat.

Shortness of breath during exercise.Shortness of breath during exercise.

Less common symptoms of severe thalassemia include:Less common symptoms of severe thalassemia include:

Headache.Headache.

Belly pain.Belly pain. Ringing in the ears.Ringing in the ears.

Chest pain.Chest pain.

A slight fever.A slight fever.

A sore, smooth tongue.A sore, smooth tongue.

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Children with a more severe form of thalassemiaChildren with a more severe form of thalassemia

(beta thalassemia major, or Cooley's anemia) usually(beta thalassemia major, or Cooley's anemia) usually

develop symptoms ofdevelop symptoms ofanemiaanemia within the first fewwithin the first few

months of life. Paler skin is often the first sign of themonths of life. Paler skin is often the first sign of the

disease.disease.22 Infants may grow slowly (Infants may grow slowly (failure to thrivefailure to thrive).).

Other symptoms may include feeding problems,Other symptoms may include feeding problems,

frequent fevers, and diarrhea. Without early treatment,frequent fevers, and diarrhea. Without early treatment,

a child may die or develop severe problems, such as:a child may die or develop severe problems, such as:33

A deformed face caused by theA deformed face caused by thebone marrowbone marrow

expanding in the bones. This may cause a bulgingexpanding in the bones. This may cause a bulgingforehead (frontal bossing).forehead (frontal bossing).

An enlargedAn enlarged liverliverandand spleenspleen..

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Patient with ThalassemiaPatient with Thalassemia

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TreatmentTreatmentBlood TransfusionsBlood Transfusions

The most common treatment for all major forms of thalassemia isThe most common treatment for all major forms of thalassemia is red blood cell transfusionsred blood cell transfusions..These transfusions are necessary to provide the patient with a temporary supply of healthy redThese transfusions are necessary to provide the patient with a temporary supply of healthy redblood cells with normal hemoglobin capable of carrying the oxygen that the patient's bodyblood cells with normal hemoglobin capable of carrying the oxygen that the patient's bodyneeds.needs.

While thalassemia patients were given infrequent transfusions in the past, clinical research ledWhile thalassemia patients were given infrequent transfusions in the past, clinical research ledto a more frequent program of regular blood cell transfusions that has greatly improved theto a more frequent program of regular blood cell transfusions that has greatly improved thepatients' quality of life. Today, most patients with a major form of thalassemia receive redpatients' quality of life. Today, most patients with a major form of thalassemia receive redblood cell transfusions every two to three weeks, amounting to as much as 52 pints of blood ablood cell transfusions every two to three weeks, amounting to as much as 52 pints of blood a

year.year.

Iron OverloadIron Overload

Because there is no natural way for the body to eliminate iron, the iron in the transfused bloodBecause there is no natural way for the body to eliminate iron, the iron in the transfused bloodcells builds up in a condition known as "iron overload" and becomes toxic to tissues andcells builds up in a condition known as "iron overload" and becomes toxic to tissues andorgans, particularly the liver and heart. Iron overload typically results in the patient's earlyorgans, particularly the liver and heart. Iron overload typically results in the patient's earlydeath from organ failure.death from organ failure.

Chelation TherapyChelation Therapy

To help remove excess iron, patients undergo "iron chelation therapy," in which a drug isTo help remove excess iron, patients undergo "iron chelation therapy," in which a drug isintroduce dinto the body which binds with excess iron and removes it through the urine orintroduce dinto the body which binds with excess iron and removes it through the urine orstool. For many years, the only FDAstool. For many years, the only FDA--approved iron chelator wasapproved iron chelator was DesferalDesferal, which has to be, which has to beadministered through a painful and difficult infusion process. When using Desferal, a needleadministered through a painful and difficult infusion process. When using Desferal, a needleis attached to a small batteryis attached to a small battery--operated infusion pump and worn under the skin of the stomachoperated infusion pump and worn under the skin of the stomachor legs five to seven times a week for up to twelve hours.or legs five to seven times a week for up to twelve hours.

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Immune Hemolytic AnemiaImmune Hemolytic Anemia

Immune hemolytic anemia is a disorder characterized byImmune hemolytic anemia is a disorder characterized byanemiaanemia due to premature destruction of red blood cells by thedue to premature destruction of red blood cells by theimmune system.immune system.

SymptomsSymptoms

FatigueFatigue

Pale orPale oryellowyellow skin colorskin color

Shortness of breathShortness of breath

Rapid heart rateRapid heart rate Dark urineDark urine

Enlarged spleenEnlarged spleen

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TreatmentTreatment

Treatment with theTreatment with the steroidsteroidprednisone is the firstprednisone is the firsttherapy that is tried. If prednisone does not improvetherapy that is tried. If prednisone does not improve

the condition,the condition, aa splenectomysplenectomy (removal of the spleen)(removal of the spleen)

may be considered. Treatments with drugs thatmay be considered. Treatments with drugs that

suppress thesuppress the immune systemimmune system will be given if thewill be given if theperson does not respond to prednisone andperson does not respond to prednisone and

splenectomy.splenectomy.

BloodBlood transfusions, if indicated for severe anemia, aretransfusions, if indicated for severe anemia, are

given with caution because of the potential thatgiven with caution because of the potential thatbloodbloodmay not be compatible and it may precipitate amay not be compatible and it may precipitate a

reaction.reaction.

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HereditaryHemocromatosisHereditaryHemocromatosis

Hereditary hemochromatosis (HH), the mostHereditary hemochromatosis (HH), the most

common form of iron overload disease, is ancommon form of iron overload disease, is an

inherited disorder that causes the body toinherited disorder that causes the body to

absorb and store too much iron. Excess iron isabsorb and store too much iron. Excess iron is

stored throughout the body in organs andstored throughout the body in organs and

tissues including the pancreas, liver, and skin.tissues including the pancreas, liver, and skin.

Without treatment, the iron deposits canWithout treatment, the iron deposits candamage these organs and tissues.damage these organs and tissues.

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Some people who test positive for hereditary hemochromatosis remainSome people who test positive for hereditary hemochromatosis remainsymptomsymptom--free for life. Kids who test positive rarely have any symptomsfree for life. Kids who test positive rarely have any symptoms

because iron takes years to accumulate.because iron takes years to accumulate. Patients who do have symptoms may experience:Patients who do have symptoms may experience:

muscle aches and joint pain, primarily in the fingers, knees, hips, andmuscle aches and joint pain, primarily in the fingers, knees, hips, andankles; one of the earliest symptoms is arthritis of the knuckles of the firstankles; one of the earliest symptoms is arthritis of the knuckles of the firstand second fingersand second fingers

chronic fatiguechronic fatigue

depression, disorientation, or memory problemsdepression, disorientation, or memory problems stomach swelling, abdominal pain, diarrhea, or nauseastomach swelling, abdominal pain, diarrhea, or nausea

loss of body hair, other than that on the scalploss of body hair, other than that on the scalp

premature menopausepremature menopause

gray or bronze skin similar to a suntangray or bronze skin similar to a suntan

heart problemsheart problems

diabetesdiabetes

enlarged liverenlarged liver

increased susceptibility to bacterial infectionsincreased susceptibility to bacterial infections

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With such a wide range of possible symptoms, theWith such a wide range of possible symptoms, the

disease can be extremely difficult to diagnose. Asdisease can be extremely difficult to diagnose. As

symptoms progress, it's frequently misdiagnosed assymptoms progress, it's frequently misdiagnosed aschronic hepatitis, other forms of diabetes, Alzheimer'schronic hepatitis, other forms of diabetes, Alzheimer's

disease, iron deficiency, gallbladder illness, menstrualdisease, iron deficiency, gallbladder illness, menstrual

problems, thyroid conditions, or polycythemia (anproblems, thyroid conditions, or polycythemia (an

increase in the number of red blood cells).increase in the number of red blood cells).

It's important to understand that someone withIt's important to understand that someone with

hereditary hemochromatosis can have somehereditary hemochromatosis can have some

symptoms without having all of them (i.e., heartsymptoms without having all of them (i.e., heartproblems without skin color changes,problems without skin color changes, diabetesdiabetes, or, or

liver problems).liver problems).

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TreatmentTreatment

Treatment is simple, inexpensive, and safe. The first step is to rid the body ofTreatment is simple, inexpensive, and safe. The first step is to rid the body ofexcess iron. The process is called phlebotomy, which means removing blood.excess iron. The process is called phlebotomy, which means removing blood.Depending on how severe the iron overload is, a pint of blood is taken once orDepending on how severe the iron overload is, a pint of blood is taken once ortwice a week for several months to a year, occasionally longer. Blood ferritin levelstwice a week for several months to a year, occasionally longer. Blood ferritin levelsare tested after every four phlebotomies to monitor iron levels. The goal is to bringare tested after every four phlebotomies to monitor iron levels. The goal is to bringblood ferritin levels to the low end of normal and keep them there (that means lessblood ferritin levels to the low end of normal and keep them there (that means lessthan 9 to 50 micrograms of blood ferritin per liter). Depending on the amount ofthan 9 to 50 micrograms of blood ferritin per liter). Depending on the amount ofoverload at diagnosis, reaching normal levels can take up to 100 phlebotomies.overload at diagnosis, reaching normal levels can take up to 100 phlebotomies.

Once iron levels return to normal, maintenance therapy, which involves removing aOnce iron levels return to normal, maintenance therapy, which involves removing apint of blood every 1 to 4 months for life, begins. Some people may need it morepint of blood every 1 to 4 months for life, begins. Some people may need it more

often. An annual blood ferritin test will help determine how often blood should beoften. An annual blood ferritin test will help determine how often blood should beremoved.removed.

The earlier hemochromatosis is diagnosed and treated, the better. If treatmentThe earlier hemochromatosis is diagnosed and treated, the better. If treatmentbegins before any organs are damaged, associated conditions such as liver disease,begins before any organs are damaged, associated conditions such as liver disease,heart disease, arthritis, and diabetes can be prevented. Individuals who have alreadyheart disease, arthritis, and diabetes can be prevented. Individuals who have alreadydeveloped complications from hemochromatosis may not be cured but usually candeveloped complications from hemochromatosis may not be cured but usually canbe helped. The main exception is arthritis, which does not improve even afterbe helped. The main exception is arthritis, which does not improve even after

excess iron is removed.excess iron is removed. People with hemochromatosis should not take iron supplements. Those who havePeople with hemochromatosis should not take iron supplements. Those who have

liver damage should not drink alcoholic beverages because they further damage theliver damage should not drink alcoholic beverages because they further damage theliver.liver.

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PolycythemiaPolycythemia

(orpolycythaemia or erythrocytosis)(orpolycythaemia or erythrocytosis)

is a condition in which there is a net increase inis a condition in which there is a net increase in

the total number ofthe total number ofblood cellsblood cells, primarily, primarily redred

blood cellsblood cells, in the body., in the body.[1][1] The overproductionThe overproduction

of red blood cells may be due to a primaryof red blood cells may be due to a primary

process in theprocess in thebone marrowbone marrow (a so(a so--calledcalled

myeloproliferative syndromemyeloproliferative syndrome), or it may be a), or it may be a

reaction to chronicallyreaction to chronically low oxygen levelslow oxygen levels or,or,rarely, ararely, a malignancymalignancy..

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The list of signs and symptoms mentioned in various sources for PolycythemiaThe list of signs and symptoms mentioned in various sources for Polycythemiaincludes the 14 symptoms listed below:includes the 14 symptoms listed below:

No symptomsNo symptoms -- in some patientsin some patients

HeadacheHeadache TirednessTiredness

BreathlessnessBreathlessness

Disturbed visionDisturbed vision

WeaknessWeakness

DizzinessDizziness General itchingGeneral itching

FlushingFlushing

Facial rednessFacial redness

Bloodshot eyesBloodshot eyes

Enlarged spleenEnlarged spleen

Bleeding spleenBleeding spleen Enlarged liverEnlarged liver

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Nursing management:Nursing management:

--Risk factors for thrombotic complications should beRisk factors for thrombotic complications should be

assessed, and patients should be instructed regardingassessed, and patients should be instructed regarding

the sings and symptoms of thrombosis.the sings and symptoms of thrombosis.

--patient with a history of bleeding are usuallypatient with a history of bleeding are usually

advised to avoid aspirin.advised to avoid aspirin.

--minimize alcohol intake should also be emphasizedminimize alcohol intake should also be emphasizedto further diminish any risk for bleeding.to further diminish any risk for bleeding.

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Patient with PolycythemiaPatient with Polycythemia

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LeukopeniaLeukopenia

(orleukocytopenia, orleucopenia)(orleukocytopenia, orleucopenia)

is a decrease in the number of circulatingis a decrease in the number of circulating whitewhite

blood cellsblood cells (leukocytes) in the(leukocytes) in thebloodblood. As the. As the

principal function of white cells is to combatprincipal function of white cells is to combat

infectioninfection, a decrease in the number of these, a decrease in the number of these

cells can place patients at increased risk forcells can place patients at increased risk for

infectioninfection..

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The first indications of leukemia often are nonspecific orThe first indications of leukemia often are nonspecific orvague. They may occur with other cancerous as well asvague. They may occur with other cancerous as well as

noncancerous disorders. Although signs and symptoms varynoncancerous disorders. Although signs and symptoms varyfor each type of leukemia, there are some general features.for each type of leukemia, there are some general features.Broad symptoms of leukemia may include:Broad symptoms of leukemia may include:

FatigueFatigue

Malaise (vague feeling of bodily discomfort)Malaise (vague feeling of bodily discomfort)

Abnormal bleedingAbnormal bleeding Excessive bruisingExcessive bruising

WeaknessWeakness

Reduced exercise toleranceReduced exercise tolerance

Weight lossWeight loss

Bone or joint painBone or joint pain Infection and feverInfection and fever

Abdominal pain or "fullness"Abdominal pain or "fullness"

Enlarged spleen, lymph nodes, and liverEnlarged spleen, lymph nodes, and liver

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Patient with LeukopeniaPatient with Leukopenia

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LeukemiaLeukemia

-- cancer of the blood cells. It starts in the bone marrow, the soft tissue insidecancer of the blood cells. It starts in the bone marrow, the soft tissue insidemost bones. Bone marrow is where blood cells are made.most bones. Bone marrow is where blood cells are made.

When you are healthy, your bone marrow makes:When you are healthy, your bone marrow makes:

White blood cellsWhite blood cells, which help your body fight infection., which help your body fight infection.

Red blood cellsRed blood cells, which carry oxygen to all parts of your body., which carry oxygen to all parts of your body. PlateletsPlatelets, which help your blood clot., which help your blood clot.

When you have leukemia, the bone marrow starts to make a lot ofWhen you have leukemia, the bone marrow starts to make a lot ofabnormal white blood cells, called leukemia cells. They don't do the workabnormal white blood cells, called leukemia cells. They don't do the workof normal white blood cells, they grow faster than normal cells, and theyof normal white blood cells, they grow faster than normal cells, and theydon't stop growing when they should.don't stop growing when they should.

Over time, leukemia cells can crowd out the normal blood cells. This canOver time, leukemia cells can crowd out the normal blood cells. This canlead to serious problems such aslead to serious problems such as anemiaanemia, bleeding, and infections., bleeding, and infections.Leukemia cells can also spread to theLeukemia cells can also spread to the lymph nodeslymph nodes or other organs andor other organs andcause swelling or pain.cause swelling or pain.

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Fevers and night sweats.Fevers and night sweats.

Frequent or unusual infections.Frequent or unusual infections.

Weakness and fatigue.Weakness and fatigue.

Headaches.Headaches.

Bruising of the skin and bleeding from the gums or rectum.Bruising of the skin and bleeding from the gums or rectum. Bone pain.Bone pain.

Joint pain.Joint pain.

Swelling in the belly or pain on the left side of the belly or inSwelling in the belly or pain on the left side of the belly or inthe left shoulder from a swollenthe left shoulder from a swollen spleenspleen..

SwollenSwollen lymph nodeslymph nodes in the armpit, neck, or groin.in the armpit, neck, or groin.

Decreased appetite and weight loss because you feel full andDecreased appetite and weight loss because you feel full anddon't want to eat.don't want to eat.

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TreatmentTreatment

ChemotherapyChemotherapy, which uses powerful medicines to kill cancer, which uses powerful medicines to kill cancercells. This is the main treatment for most types of leukemia.cells. This is the main treatment for most types of leukemia.

Radiation treatmentsRadiation treatments. Radiation therapy uses high. Radiation therapy uses high--dose Xdose X--raysraysto destroy cancer cells and shrink swollen lymph nodes or anto destroy cancer cells and shrink swollen lymph nodes or an

enlarged spleen. It may also be used before a stem cellenlarged spleen. It may also be used before a stem celltransplant.transplant.

Stem cell transplantStem cell transplant. Donated stem cells can rebuild your. Donated stem cells can rebuild yoursupply of normal blood cells and boost your immune system.supply of normal blood cells and boost your immune system.Before the transplant, radiation or chemotherapy is used toBefore the transplant, radiation or chemotherapy is used to

destroy cells in bone marrow and make room for donated cells.destroy cells in bone marrow and make room for donated cells. Biological therapyBiological therapy. This is the use of special medicines that. This is the use of special medicines that

improve your body's natural defenses against cancerimprove your body's natural defenses against cancer

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Patient with LeukemiaPatient with Leukemia

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Agnogenic myeloid metaplasiaAgnogenic myeloid metaplasia

-- a condition that occurs when the bone marrow isa condition that occurs when the bone marrow isscarred, making it less likely to manufacture bloodscarred, making it less likely to manufacture bloodcells. When the bone marrow does not functioncells. When the bone marrow does not functionproperly, theproperly, the spleenspleen andand liverliverwill produce blood cells.will produce blood cells.As a result, the spleen and liver may become enlarged.As a result, the spleen and liver may become enlarged.

Agnogenic myeloid metaplasia usually developsAgnogenic myeloid metaplasia usually developsslowly. It most often occurs in people 50 years oldslowly. It most often occurs in people 50 years oldand older. Agnogenic myeloid metaplasia leads toand older. Agnogenic myeloid metaplasia leads toprogressive bone marrow failure with severe anemia.progressive bone marrow failure with severe anemia.

Most people with agnogenic myeloid metaplasiaMost people with agnogenic myeloid metaplasiabecome dependent on blood transfusions for survival.become dependent on blood transfusions for survival.

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Signs and SypmtomsSigns and Sypmtoms

rapid heart beatrapid heart beat

palenesspaleness

enlarged spleenenlarged spleen night sweatsnight sweats

weight lossweight loss

stuffed feeling after eatingstuffed feeling after eating

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TreatmentTreatment

Only bone marrow transplantation has been shown to cure myelofibrosis.Only bone marrow transplantation has been shown to cure myelofibrosis.But other treatments can improve symptoms of the disease. Decreases inBut other treatments can improve symptoms of the disease. Decreases inred and white blood cells and platelets can be treated with androgens,red and white blood cells and platelets can be treated with androgens,erythropoietin and thalidomide. An enlarged spleen can be helped in someerythropoietin and thalidomide. An enlarged spleen can be helped in somecases with surgery, oralcases with surgery, oral chemotherapychemotherapy with hydroxyurea or lowwith hydroxyurea or low--dosedoseradiationradiation. These treatments have side effects and must be carefully. These treatments have side effects and must be carefully

managed so that the benefits outweigh the risks.managed so that the benefits outweigh the risks. Mayo physicians have significant clinical expertise in using currentlyMayo physicians have significant clinical expertise in using currently

available medications to treat myelofibrosis. Many clinical trials ofavailable medications to treat myelofibrosis. Many clinical trials ofmedications have been performed at Mayo, including Suramin, Pirfenidone,medications have been performed at Mayo, including Suramin, Pirfenidone,InterferonInterferon--Alpha, Etanercept, Thalidomide, Thalidomide plus Prednisone,Alpha, Etanercept, Thalidomide, Thalidomide plus Prednisone,Imatinib Mesylate (Gleevec), Cladribine and Zarnestra. Additionally, MayoImatinib Mesylate (Gleevec), Cladribine and Zarnestra. Additionally, Mayoresearchers (seeresearchers (see Research on MyelofibrosisResearch on Myelofibrosis) are actively evaluating) are actively evaluatingpossible new therapies for myelofibrosis in the laboratory. Many clinicalpossible new therapies for myelofibrosis in the laboratory. Many clinicaltrials are currently under way for myelofibrosis and related diseases.trials are currently under way for myelofibrosis and related diseases.

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Patient withPatient with Agnogenic myeloidAgnogenic myeloid

metaplasiametaplasia

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LymphomaLymphoma

is a type of solidis a type of solid neoplasmneoplasm that originates inthat originates in

lymphocyteslymphocytes (a type of(a type ofwhite blood cellwhite blood cell in thein the

vertebratevertebrate immune systemimmune system). This is in contrast). This is in contrast

toto lymphoid leukemialymphoid leukemia, which is a malignancy, which is a malignancy

of circulating cells.of circulating cells.[1][1]

There are many types of lymphoma.There are many types of lymphoma.

Lymphomas are part of the broad group ofLymphomas are part of the broad group ofdiseases calleddiseases called hematological neoplasmshematological neoplasms

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Painless lymph node enlargementPainless lymph node enlargement

Fevers, night sweats, tiredness, weight loss >10%Fevers, night sweats, tiredness, weight loss >10%

Widespread itchingWidespread itching

Nausea, vomiting, abdominal painNausea, vomiting, abdominal pain Shortness of breath, coughShortness of breath, cough

Headaches, vision changes, seizuresHeadaches, vision changes, seizures

AnaemiaAnaemia

Susceptible to infections eg shinglesSusceptible to infections eg shingles

Reddened patches on the skinReddened patches on the skin

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Pain ManagementPain Management

Pain relief is not the only goal of pain management. It is alsoPain relief is not the only goal of pain management. It is alsointended to help you maintain your normal quality of lifeintended to help you maintain your normal quality of lifethroughout your lymphoma treatment. At CTCA, your carethroughout your lymphoma treatment. At CTCA, your careteam will incorporate various pain management techniquesteam will incorporate various pain management techniques

into your lymphoma cancer treatment plan. All methods ofinto your lymphoma cancer treatment plan. All methods ofpain management attempt to either control the cause of thepain management attempt to either control the cause of thepain or alter your perception of it.pain or alter your perception of it.

Although there are several different pain managementAlthough there are several different pain managementtechniques, therapeutic approaches can be classified as eithertechniques, therapeutic approaches can be classified as either

pharmacological or nonpharmacological or non--pharmacological. Pharmacologicalpharmacological. Pharmacologicalpain control involves the use of pain medications (analgesics),pain control involves the use of pain medications (analgesics),along with other medications that either intensify thealong with other medications that either intensify theanalgesics' effects or modify your mood or pain perception.analgesics' effects or modify your mood or pain perception.

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The following are some nonThe following are some non--pharmacologicalpharmacological

approaches to pain management:approaches to pain management:

Behavioral techniquesBehavioral techniques

Emotional counseling and supportEmotional counseling and support

Radiation to shrink the tumorRadiation to shrink the tumor Surgery to remove part or all of the tumorSurgery to remove part or all of the tumor

Neurological and neurosurgical interventionsNeurological and neurosurgical interventions

Traditional nursing and psychosocial interventionsTraditional nursing and psychosocial interventions

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Multiple myelomaMultiple myeloma(plasma cell myeloma(plasma cell myeloma

Kahler's disease )Kahler's disease )is a type ofis a type ofcancercancerofofplasma cellsplasma cells which arewhich are

immune systemimmune system cells in bone marrow thatcells in bone marrow that

produceproduce antibodiesantibodies. Myeloma is regarded as. Myeloma is regarded as

incurable, but remissions may be induced withincurable, but remissions may be induced with

steroidssteroids,, chemotherapychemotherapy,, thalidomidethalidomide andand stemstem

cell transplantscell transplants. Myeloma is part of the broad. Myeloma is part of the broad

group of diseases calledgroup of diseases called hematologicalhematologicalmalignanciesmalignancies..

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Because many organs can be affected by myeloma, theBecause many organs can be affected by myeloma, thesymptoms and signs vary greatly. Asymptoms and signs vary greatly. A mnemonicmnemonic sometimessometimesused to remember the common tetrad of multiple myeloma isused to remember the common tetrad of multiple myeloma isCRABCRAB -- C = Calcium (elevated), R = Renal failure, A =C = Calcium (elevated), R = Renal failure, A =

Anemia, B = Bone lesions.Anemia, B = Bone lesions.[1][1] Myeloma has many possibleMyeloma has many possiblesymptoms, and all symptoms may be due to other causes. Theysymptoms, and all symptoms may be due to other causes. Theyare presented here in decreasing order of incidence.are presented here in decreasing order of incidence.

Bone pain, often in the back or ribsBone pain, often in the back or ribs

Broken bonesBroken bones

Weakness or fatigueWeakness or fatigue

Weight lossWeight loss

Repeated infectionsRepeated infections

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TreatmentTreatment

Myeloma is not yet considered curable, but is anMyeloma is not yet considered curable, but is anincreasingly treatable condition. Treatments areincreasingly treatable condition. Treatments arefocused on:focused on:

controlling the diseasecontrolling the disease

prolonging survivalprolonging survival improving symptoms so that patients can live withimproving symptoms so that patients can live with

myeloma and have the best possible quality of life.myeloma and have the best possible quality of life.

In addition to direct treatment of the plasma cellIn addition to direct treatment of the plasma cell

proliferation,proliferation,bisphosphonatesbisphosphonates (e.g.(e.g.pamidronatepamidronate ororzoledronic acidzoledronic acid) are routinely administered to prevent) are routinely administered to preventfractures andfractures and erythropoietinerythropoietin to treat anemia.to treat anemia.

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Bleeding AnemiaBleeding Anemia

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Primary thrombocythemiaPrimary thrombocythemia

Primary thrombocythemia is thePrimary thrombocythemia is the

overproduction of platelets without aoverproduction of platelets without a

recognizable cause. Platelets are essential forrecognizable cause. Platelets are essential for

blood clotting.blood clotting.

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Bleeding from the gastrointestinal tractBleeding from the gastrointestinal tract

Bleeding from the gumsBleeding from the gums

Bloody stoolsBloody stools

DizzinessDizziness

EasyEasybruisingbruising Enlarged lymph nodesEnlarged lymph nodes (rare)(rare)

HeadacheHeadache

Nosebleeds (Nosebleeds (epistaxisepistaxis))

Numbness of hands or feetNumbness of hands or feet

Prolonged bleeding from surgical procedures or toothProlonged bleeding from surgical procedures or toothextractionextraction

Ulcers on fingers or toesUlcers on fingers or toes

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Nursing managementNursing management

Patients with primary thrombocythemia need tobePatients with primary thrombocythemia need tobeinstructed about the accompanying risks of hemorrhageinstructed about the accompanying risks of hemorrhageand thrombosis.The patient is informed about signs andand thrombosis.The patient is informed about signs andsymptoms of thrombosis, particularly the neurologicsymptoms of thrombosis, particularly the neurologicnn\\manifestations, such as visual changes, numbness,manifestations, such as visual changes, numbness,

tingling, and weakness. Risk factors for thrombosis aretingling, and weakness. Risk factors for thrombosis areassessed, and measures to diminish risk factors areassessed, and measures to diminish risk factors areencouraged.Patients receiving aspirin therapy should beencouraged.Patients receiving aspirin therapy should beinformed about the increased riskofbleeding.Patientsinformed about the increased riskofbleeding.Patientswho are at risk forbleeding should be instructed aboutwho are at risk forbleeding should be instructed aboutmedications ( eg, aspirin, NSAIDs ) and other substancesmedications ( eg, aspirin, NSAIDs ) and other substances( eg, alcohol ) that can alterplatelet function.Patients( eg, alcohol ) that can alterplatelet function.Patientsreceiving interferon therapy are taught to selfreceiving interferon therapy are taught to self--administeradministerthe medication and manage side effectsthe medication and manage side effects

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Patient with ThrombocythemiaPatient with Thrombocythemia

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THROMBOCYTOSISTHROMBOCYTOSIS

(SecondaryThrombocythemia)(SecondaryThrombocythemia)Thrombocytosis can develop secondary to chronic inflammatoryThrombocytosis can develop secondary to chronic inflammatory

disorders, acute infection, hemorrhage, iron deficiency,disorders, acute infection, hemorrhage, iron deficiency,hemolysis, or tumors. Platelet function is usually normal.hemolysis, or tumors. Platelet function is usually normal.However, in myeloproliferative disorders, abnormalities ofHowever, in myeloproliferative disorders, abnormalities of

platelet aggregation occur in about 50% of patients. Unlike ET,platelet aggregation occur in about 50% of patients. Unlike ET,thrombocytosis does not increase the risk of thrombotic orthrombocytosis does not increase the risk of thrombotic orhemorrhagic complications unless patients have severe arterialhemorrhagic complications unless patients have severe arterialdisease or prolonged immobility. With secondarydisease or prolonged immobility. With secondarythrombocytosis, the platelet count is usually < 1,000,000/L,thrombocytosis, the platelet count is usually < 1,000,000/L,

and the cause may be obvious from the history, physicaland the cause may be obvious from the history, physicalexamination, or radiologic or blood testing. Treatment of theexamination, or radiologic or blood testing. Treatment of theunderlying disorder usually returns the platelet count to normal.underlying disorder usually returns the platelet count to normal.

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High platelet levels do not necessarily signal any clinicalHigh platelet levels do not necessarily signal any clinicalproblems, and are picked up on a routineproblems, and are picked up on a routine full blood countfull blood count..However, it is important that a full medical history be elicitedHowever, it is important that a full medical history be elicitedto ensure that the increased platelet count is not due to ato ensure that the increased platelet count is not due to asecondary process. Often, it occurs in tandem with ansecondary process. Often, it occurs in tandem with an

inflammatory diseaseinflammatory disease, as the principal stimulants of platelet, as the principal stimulants of plateletproduction (e.g.production (e.g. thrombopoietinthrombopoietin) are elevated in these clinical) are elevated in these clinicalstates as part of thestates as part of the acute phase reactionacute phase reaction..

High platelet counts can occur in patients withHigh platelet counts can occur in patients withpolycythemiapolycythemiaveravera (high(high red blood cellred blood cell counts), and is an additional riskcounts), and is an additional risk

factor for complications.factor for complications. A very small segment of patients report symptoms ofA very small segment of patients report symptoms of

erythromelalgiaerythromelalgia, a burning sensation and redness of the, a burning sensation and redness of theextremities that resolves with cooling and/orextremities that resolves with cooling and/oraspirinaspirin use.use.

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Patient withPatient with SecondarySecondary

ThrombocythemiaThrombocythemia

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ThrombocytopeniaThrombocytopenia

Thrombocytopenia (orThrombocytopenia (or --paenia, or thrombopenia inpaenia, or thrombopenia inshort) is the presence of relatively fewshort) is the presence of relatively fewplateletsplatelets ininbloodblood..

Generally speaking, in humans, a normal plateletGenerally speaking, in humans, a normal platelet

count ranges from 150,000 and 450,000 per mm3count ranges from 150,000 and 450,000 per mm3(microlitre).(microlitre).[1][1] These limits, however, are determinedThese limits, however, are determinedby the 2.5th lower and upperby the 2.5th lower and upperpercentilepercentile, and a, and adeviation does not necessarily imply any form ofdeviation does not necessarily imply any form ofdisease. The number of platelets in a blood sampledisease. The number of platelets in a blood sample

also decreases rather quickly with time and a lowalso decreases rather quickly with time and a lowplatelet count may be caused by a delay betweenplatelet count may be caused by a delay betweensampling and analysis.sampling and analysis.

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Easy or excessive bruisingEasy or excessive bruising

Superficial bleeding into the skin that appears as aSuperficial bleeding into the skin that appears as a

rash of pinpointrash of pinpoint--sized reddishsized reddish--purple spotspurple spots

(petechiae), usually on the lower legs(petechiae), usually on the lower legs Prolonged bleeding from cutsProlonged bleeding from cuts

Spontaneous bleeding from your gums or noseSpontaneous bleeding from your gums or nose

Blood in urine or stoolsBlood in urine or stools Unusually heavy menstrual flowsUnusually heavy menstrual flows

Profuse bleeding during surgeryProfuse bleeding during surgery

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TreatmentTreatment

If your platelet count is low, your doctor may recommend avoiding drugsIf your platelet count is low, your doctor may recommend avoiding drugssuch as aspirin, which may impair platelet function, and avoiding excessivesuch as aspirin, which may impair platelet function, and avoiding excessivealcohol intake. You may also wish to avoid contact sports, which can putalcohol intake. You may also wish to avoid contact sports, which can putyou at a higher risk of injury and bleeding.you at a higher risk of injury and bleeding.

Sometimes, treatment for thrombocytopenia isn't necessary, particularly inSometimes, treatment for thrombocytopenia isn't necessary, particularly inchildren. In these cases, the bone marrow may make up for the shortage ofchildren. In these cases, the bone marrow may make up for the shortage of

platelets by producing large numbers of new ones until the initiating causeplatelets by producing large numbers of new ones until the initiating causesubsides. Young platelets are especially active in clotting, so even thoughsubsides. Young platelets are especially active in clotting, so even thoughthe total concentration of platelets is low, your child may not experiencethe total concentration of platelets is low, your child may not experienceany bleeding problems and need no special treatment.any bleeding problems and need no special treatment.

Mild thrombocytopenia in pregnant women usually improves soon afterMild thrombocytopenia in pregnant women usually improves soon afterchildbirth.childbirth.

If your doctor can identify the cause of thrombocytopenia, then theIf your doctor can identify the cause of thrombocytopenia, then theapproach is to treat the underlying condition or stop using the problemapproach is to treat the underlying condition or stop using the problemmedication.medication.

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Treating thrombocytopenia may involve several options:Treating thrombocytopenia may involve several options:

Medications. For idiopathic thrombocytopenic purpura, treatment mayMedications. For idiopathic thrombocytopenic purpura, treatment mayinclude medications that block the antibodies that attack platelets, such asinclude medications that block the antibodies that attack platelets, such as

corticosteroids. Intravenous immunoglobulin may be used when rapidcorticosteroids. Intravenous immunoglobulin may be used when rapidelevation of your platelet count is needed. If corticosteroids don't help, yourelevation of your platelet count is needed. If corticosteroids don't help, yourdoctor may recommend medications that suppress your immune system todoctor may recommend medications that suppress your immune system toreduce antibody formation, such as cyclophosphamide (Cytoxan) orreduce antibody formation, such as cyclophosphamide (Cytoxan) orazathioprine (Imuran).azathioprine (Imuran).

Surgery. Sometimes, removing the spleen (splenectomy) relieves signs andSurgery. Sometimes, removing the spleen (splenectomy) relieves signs andsymptoms or helps improve chronic idiopathic thrombocytopenic purpurasymptoms or helps improve chronic idiopathic thrombocytopenic purpurathat doesn't respond to corticosteroids.that doesn't respond to corticosteroids.

Blood transfusions and plasma exchange. For severe bleeding, your doctorBlood transfusions and plasma exchange. For severe bleeding, your doctorcan replace lost blood with transfusions of packed red blood cells. Plateletcan replace lost blood with transfusions of packed red blood cells. Plateletconcentrates are given to treat severe thrombocytopenia, particularly thoseconcentrates are given to treat severe thrombocytopenia, particularly thoserelated to cancer or chemotherapy.related to cancer or chemotherapy.

Thrombotic thrombocytopenic purpura requires emergency treatment withThrombotic thrombocytopenic purpura requires emergency treatment with

plasma exchange therapy or therapeutic plasmapheresis. If you haveplasma exchange therapy or therapeutic plasmapheresis. If you havehemolytic uremic syndrome, you may need kidney dialysis in addition tohemolytic uremic syndrome, you may need kidney dialysis in addition tored blood cell and plateletred blood cell and platelet

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Patient with ThrombocytopeniaPatient with Thrombocytopenia

Idi hi h b iIdi hi h b i

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Idiopathic thrombocytopenicIdiopathic thrombocytopenic

purpura (ITP)purpura (ITP) Idiopathic thrombocytopenic purpura (ITP), also called immuneIdiopathic thrombocytopenic purpura (ITP), also called immune

thrombocytopenic purpura, is a bleeding disorder in which your blood hasthrombocytopenic purpura, is a bleeding disorder in which your blood hasdifficulty clotting due to an unusually low number of platelets. Plateletsdifficulty clotting due to an unusually low number of platelets. Platelets(thrombocytes) are colorless blood cells that stop blood loss by clumping(thrombocytes) are colorless blood cells that stop blood loss by clumpingtogether at the site of a blood vessel injury and forming plugs in vesseltogether at the site of a blood vessel injury and forming plugs in vesselholes.holes.

People with idiopathic thrombocytopenic purpura, because of their lowPeople with idiopathic thrombocytopenic purpura, because of their lowplatelet count, tend to bruise easily and bleed longer when injured.platelet count, tend to bruise easily and bleed longer when injured.Nosebleeds and bleeding gums also are common.Nosebleeds and bleeding gums also are common.

Idiopathic thrombocytopenic purpura is often divided into two categories:Idiopathic thrombocytopenic purpura is often divided into two categories:

acute and chronic. Acute ITP is the most common form and occurs mostacute and chronic. Acute ITP is the most common form and occurs mostfrequently in children, typically after a viral infection. It usually goes awayfrequently in children, typically after a viral infection. It usually goes awayon its own within six months. Chronic ITP lasts longer than six months andon its own within six months. Chronic ITP lasts longer than six months andis more common in adults.is more common in adults.

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BruisingBruising

Nosebleed or bleeding in the mouthNosebleed or bleeding in the mouth Bleeding into the skinBleeding into the skin -- also called pinpointalso called pinpoint

red spots or petechial rashred spots or petechial rash

Abnormally heavy menstruationAbnormally heavy menstruation

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Prehospital care focuses on the ABCs, which include providing oxygen,Prehospital care focuses on the ABCs, which include providing oxygen,controlling severe hemorrhage, and initiating intravenous (IV) fluids tocontrolling severe hemorrhage, and initiating intravenous (IV) fluids tomaintain hemodynamic stability.maintain hemodynamic stability.

Prehospital airway control maybe necessary for a large intracranialPrehospital airway control maybe necessary for a large intracranialhemorrhage.hemorrhage.

EMS

providers should be aware of the potential for serious bleedingEMS

providers should be aware of the potential for serious bleedingcomplications in patients with idiopathic thrombocytopenic purpura (ITP).complications in patients with idiopathic thrombocytopenic purpura (ITP).

LifeLife--threatening bleeding requires conventional critical care interventions.threatening bleeding requires conventional critical care interventions.

In the patient with known ITP, highIn the patient with known ITP, high--dose parenteral glucocorticoids and IVdose parenteral glucocorticoids and IVimmunoglobulin (IVIg), with or without platelet transfusions, are appropriate.immunoglobulin (IVIg), with or without platelet transfusions, are appropriate.

Platelet transfusion is indicated for controlling severe hemorrhage.Send aPlatelet transfusion is indicated for controlling severe hemorrhage.Send ablood specimen to the lab for type and screen in case platelet transfusion isblood specimen to the lab for type and screen in case platelet transfusion is

necessary.necessary.

Platelet survival is increased if the platelets are transfused immediately afterPlatelet survival is increased if the platelets are transfused immediately afterIVIg infusion.IVIg infusion.

A consultation with a hematologist maybe required to make a decisionA consultation with a hematologist maybe required to make a decisionregarding the transfusion ofplatelets.regarding the transfusion ofplatelets.

InterventionsInterventions

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Platelet defectPlatelet defect

Acquired platelet function defects are diseases orAcquired platelet function defects are diseases or

conditions that cause theconditions that cause theplateletsplatelets (the blood(the blood

cells essential for clotting) to not workcells essential for clotting) to not work

properly. The term "acquired" means theseproperly. The term "acquired" means thesediseases or conditions are not inherited, butdiseases or conditions are not inherited, but

rather develop independently in a person.rather develop independently in a person.

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SymptomsSymptoms

Abnormal color of urineAbnormal color of urine ((blood in the urineblood in the urine))

Abnormal menstrual periodsAbnormal menstrual periods Heavy menstrual periodsHeavy menstrual periods

Prolonged menstrual bleeding (more than five days per menstrual period)Prolonged menstrual bleeding (more than five days per menstrual period)

Abnormal vaginal bleedingAbnormal vaginal bleeding

Bleeding under the skin or in the muscles (soft tissues)Bleeding under the skin or in the muscles (soft tissues) Gastrointestinal bleedingGastrointestinal bleeding

Bloody, dark black, or tarry bowel movementsBloody, dark black, or tarry bowel movements

Vomiting bloodVomiting blood or vomiting material that resembles coffee groundsor vomiting material that resembles coffee grounds

NosebleedsNosebleeds

Prolonged bleeding, easyProlonged bleeding, easybruisingbruising

Skin rashSkin rash Multiple lesionsMultiple lesions

BruisesBruises

Pinpoint red spotsPinpoint red spots

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InterventionIntervention

Treatment is directed at the cause of theTreatment is directed at the cause of the

abnormality.abnormality. DialysisDialysis reduces the bleedingreduces the bleeding

tendency if the cause is renal failure. Platelettendency if the cause is renal failure. Platelet

transfusions or plateletpheresis (removal oftransfusions or plateletpheresis (removal ofplatelets from the blood and replacement withplatelets from the blood and replacement with

donated platelets) may be needed in bonedonated platelets) may be needed in bone

marrow disorders. Medicationmarrow disorders. Medication--induced plateletinduced plateletabnormalities require stopping the medicationabnormalities require stopping the medication

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Congenitalplatelet function defectsCongenitalplatelet function defects

Congenital platelet function defects are problemsCongenital platelet function defects are problems

with the blood cells that are needed for bloodwith the blood cells that are needed for blood

clotting. Congenital means present from birth.clotting. Congenital means present from birth.

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SymptomsSymptoms

NosebleedsNosebleeds

Bleeding during and after surgeryBleeding during and after surgery

Easy bruisingEasy bruising Prolonged bleeding with small injuriesProlonged bleeding with small injuries

Abnormal menstrual periodsAbnormal menstrual periods

Bleeding gumsBleeding gums

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InterventionIntervention

There is no specific treatment for theseThere is no specific treatment for these

disorders. All people with bleeding disordersdisorders. All people with bleeding disorders

should avoid taking aspirin because it isshould avoid taking aspirin because it is

known to prolong bleeding timeknown to prolong bleeding time ---- the time itthe time ittakes to form clotting mechanisms in thetakes to form clotting mechanisms in the

blood. Platelet transfusions may be given whenblood. Platelet transfusions may be given when

patients have severe bleedingpatients have severe bleeding

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