Hemolytic Anemia(Presentation 1) (2)

8/6/2019 Hemolytic Anemia(Presentation 1) (2)

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Amala

Nisanthi

Kavitha

Afiqah

Shuhaila





Is a disorder in which the red blood cells aredestroyed faster than the bone marrow can producethem

The term for destruction of red blood cells is hemolysis Iron and other by ²products remain in plasma.Lysis

within circulatory system or due to phargocytosis bycells of retiloendothetial system

Hematopoietic activities of bone marrow increase



There are 2 types of hemolytic anemiaincluding the following:

*Intrinsic-the destruction of the RBC due toa defect within the RBC themselves

Intrinsic hemolytic anemia's are ofteninherited, such as sickle cell anemia and

thalassemia

These conditions produce RBC that do notlive as long as normal RBC



Extrintic- RBC are produced healthy but

are later destroyed by becoming

trapped in the spleen,destroyed byinfection



Abnormal paleness or lack of color of thespleen

Jaundice Dark color of urine Fever Weakness Dizziness Confusion Enlargement of the spleen and liver tachycardia



Blood test

Medical history

Physical e xamination



o Change in diet

o Medication

o Splenectomy-surgery to remove thespleen









Hemoglobin is a protein inside red blood cellthat carries o xygen

Abnormal hemoglobin call hemoglobin S

Distorts the shape of red blood cells especiallywhen e xposed to low o xygen levels

Sickle cell deliver less o xygen to the body tissue

Clog more easily in small blood vessel, break into pieces that disrupt healthy blood flow

Inherited from both parents





Bone pain

Breathlessness

Fatigue Rapid heart rate

Ulcer on the lower leg(adult)

Yellowing of the eyes and skin(jaundice) Excessive thirst

Poor eye sight/blindness

Stroke





� Infection and gangrene

� Hypovolemic shock and death due tomassive entrapment of cells

� Necrosis

� Cerebral vessel occlusion due to organinfraction



CBC ²Complete Blood Count

Sickle Cell Test



Folic acid (essential for producing RBC),RBCare turned over so quickly

Narcotic ² To treat the pain(but some

people will large doses of narcotics) Kidney transplant for kidney

disease(Dialysis) Antibiotic and vaccine-prevent bacterial

infection(children with sickle cell) Large amount of [email protected]. fluids to correct

hypovolemia and prevent dehydrationvessel occlusion



� Acute pain related occlusion of small

vessel by sickle cells



� Assess pain types, location and intensity

R:Other vessel to the brain ,heart , lungs, spleen and penis becomeoccluded , causing pain

� Support joints and lower e xtremities withpillows

R:Relieve the joints pain

� Administer analgesic as ordered by doctor R: Relief from pain

� Document patient progress in nursing report R: Document pain levels



� Ineffectiveness Tissue Perfusion related to

a decreased number of RBCs



� Monitor vital sign and mental alertness R:As a baseline and monitor abnormality

� Encourage to drink 8 to 10 glasses of water daily

R: Dehydration causes RBCs to sickle cells

� Monitor for symptom of obstructed vesselssuch as leg ulcerations, and dyspnea

R: RBCs are sickling, vessels supplying blood to other organ canbecome obstucted

� Document patient progress in nursing report R: Document the abnormal of condition such as breathing pattern







� the molecular abnormalities that led to

the thalassemic syndromes, it still is not

known how accumulation of e xcessunmatched alpha-globin in beta

thalassemia.

� O xidant injury may cause hemolysis, butthere is no evidence that it causes

ineffective erythropoiesis



� Hemoglobin E/beta thalassemia is now aworldwide clinical problem. The reasons

underlying the heterogeneity andoccasional severity of the syndromeremain obscure.

� Ineffective erythropoiesis now appears

to be caused by accelerated apoptosis,in turn caused primarily by deposition ofalpha-globin chains in erythroidprecursors.



�

Heredity disorders ofhemoglobin-chain

synthesis.



1. ALPHA THALASSEMIA2. BETA THALASSEMIA



1. ALPHA THALASSEMIA� occurs when a gene or genes related to the

alpha globin protein are missing or changed(mutated).

� occur most commonly in persons fromsoutheast Asia, the Middle East, China, and inthose of African descent.

2. BETA THALASSEMIA� occurs when similar gene defects affect

production of the beta globin protein.

� occur in persons of Mediterranean origin, andto a lesser extent, Chinese, other Asians, andAfrican Americans.



� There are many forms of thalassemia.

Each type has many different subtypes.Both alpha and beta thalassemia

include the following two forms:

1. THALASSEMIA MINOR

2. THALASSEMIA MAJOR



1. THALASSEMIA MINOR� Less severe

� Receive the defective gene from only oneparent.

�

Blood smear are small(microcytic),pale(hypochromic), variouslyshape(poikilocytosis) RBC.

� Carry less o xygen.

�

Persons with this form of the disorder arecarriers of the disease and usually do nothave symptoms.



1. THALASSEMIA MAJOR

� Severe

� Inherit the defective gene from both

parents� O xygen depletion in the body becomes

apparent within the first 6 months of life.

� Left untreated, death usually results when a

few years.



� Still birth(death of the unborn baby duringbirth or the late stages of pregnancy).

� Children born with thalassemia major

(Cooley's anemia) are normal at birth, butdevelop severe anemia during the first yearof life.

� Bone deformities in the face�

Fatigue� Growth failure� Shortness of breath� Yellow skin (jaundice)



� CBC

� Iron level and total iron-binding capacity

� Serum ferritin

� Sickle cell test

� Bone marrow e xamination

� Quantitive essay of g6pd



� Assess the patient at risk for falls using a

fall risk assessment tool

� Assist the patient to change positions� Assist the patient with ambulation

� Always raise the cord side bed to

preventpatient falling down.







� Glucose-6-phosphate dehydrogenase

deficiency is an X-linked recessive

hereditary disease characterised byabnormally low levels of glucose-6-

phosphate dehydrogenase.

� Glucose-6-phosphate dehydrogenase

deficiency is a genetic disorder thatoccurs most often in males.



� G6PD deficiency reduces energy

available to maintain the integrity of the

red cell membrane, which shortens RBCsurvival.

� Hemolysis selectively affects older RBCs

among affected blacks and amongmost affected whites.



� Hemolysis occurs commonly after fever,

acute viral and bacterial infections, and

diabetic acidosis. Less commonly,hemolysis occurs after e xposure to drugs

or to other substances that produce

pero xide and cause o xidation of Hb and

RBC membranes.



�

Inherited disorders, sex-linked, RBC metabolic

disorder



� Father is UNAFFECTED, mother is UNAFFECTED.ALL of their children will be UNAFFECTED.

� Father is G6PD DEFICIENT, mother is UNAFFECTED.

All sons will be UNAFFECTED.All daughters will be PARTIALLY DEFICIENT.

� Father is G6PD DEFICIENT, mother is PARTIALLYDEFICIENT.Out of two sons, one will be G6PD DEFICIENT and the

other will be UNA

FFE

CTE

D.Out of two daughters, one will be PARTIALLYDEFICIENT and the other will be G6PD DEFICIENT.





� Paleness (in darker-skinned childrenpaleness is sometimes best seen in themouth, especially on the lips or tongue)

� Extreme tiredness

� Rapid heartbeat

� Rapid breathing or shortness breath

� Jaundice or yellowing of the skin and eyesparticularly in newborns and enlargedspleen

� Dark, tea-colored urine



� CBC

� Iron level and total iron-binding capacity

� Quantitive essay of G6PD



� Rest in bed

� Assess the patients general condition

� Adminiser o xygen as prescribed

� Refer abnormal investigation results

� Give medication as ordered by doctor



� Consult a dietition

� Administer supplements as ordered

� Administer liquid supplements with a drinkingstraw to avoid staining the teeth

� Administer intramuscular iron injection by theZ-track method to avoid staining the injectionsite



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Hemolytic Anemia(Presentation 1) (2)