Chapter III D45-D89 Diseases of the blood and
blood-forming organs
นพ.บก เจญล
กมงานลยกรรม รพ.สวรรประชาก นครสวรร
1
Documentation/Coding of Anemia
Looking for Specific Documentation Type of anemia [i.e. Acute blood loss, Aplastic anemia, Pure red cell
aplasia, Hemolytic anemia, Anemia in chronic disease] !
Review Laboratory/Pathology findings
Blood values following a surgical procedure
Significant drop of Hb/Hct !
Query the Physician for Specific diagnosis relating to these findings
Underling cause of the anemia [e.g. long-term anticoagulants, gastric
ulcer, neoplasms, internal bleeding, thalassemia]
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Complete Blood Count
Hemoglobin (g/dL) amount of oxygen carrying protein
Hematocrit (%) % of blood volume occupied by RBCs
RBC count (/mL) number of RBCs
MCV (fL) mean corpuscular (cell) volume
MCH (pg) mean corpuscular (cell) haemoglobin
MCHC (g/dL) mean corpuscular (cell) haemoglobin
concentration
RDW (%) red cells distribution width
WBC count (/mL) number of WBCs
Platelet count (/mL) number of platelets
Abnormal any Values are Not Disease, the Etiology is…..
3
Iron deficiency anemia
Anemia from acute blood loss
Anemia from chronic blood loss
Anemia in chronic disease
Anemia in neoplastic disease
Hemolytic anemia
Aplastic anemia
Thalassemia
Anemia
4
Anemia
Age/Gender group Hb threshold (g/dL) Hct (%)
Men (>15 years old) 13.0 39
Woman, non-pregnant (15 years old) 12.0 36
Woman, pregnant 11.0 33
Teens (12-15 years old) 12.0 36
Children (5-12 years old) 11.5 34
Children (0.5-5 years old) 11.0 33
WHO’s Hemoglobin thresholds used to define anemia
Anemia is present when the haemoglobin level is
more than 2SD below the mean.
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Anemia
Based on Morphology [Volume]
Normocytic - MCV 80-100 fL Acute blood loss, Aplastic anemia, Pure red cell aplasia, Hemolytic
anemia, Anemia in chronic disease, Renal failure, Marrow infiltration !
Microcytic - MCV <80 fL Iron deficiency anemia, Anemia in chronic disease (late), Thalassemia !
Macrocytic - MCV >100 fL Folate or B12 deficiency anemia, Myelodysplastic syndrome, Chronic
liver disease, Hypothyroidism, Reticulocytosis
Size Does Not Answer All Questions
Be Aware of Mixed Anemias..!!
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Anemia
Based on Etiology [Pathophysiology]
Decreased Production (Nutritional Deficiency) Iron deficiency, Folic acid and B12 deficiency, Malnutrition !
Decreased Production (Defects in Bone Marrow) Aplastic anemia, Leukemia, Lymphoma, Myeloproliferative syndrome,
Anemia in chronic disease, Infections (TB, HIV, Parvovirus) !
Blood Loss Acute or blood loss, Chronic blood loss, Hemolysis !
Increased Destruction Hemolytic anemia, G6PD deficiency, Spherocytosis, Ellipocytosis,
Thalassemia, Sickle cell disease, Paroxymal nocturnal hemolysis, DIC, TTP
Anemia is actually a sign of a disease process
rather than a disease itself.
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Normocytic Anemia
Low Reticulocyte Count (<0.5%)
Inadequate Production
High Reticulocyte Count (
Increased Destruction
Hereditary pure red cell aplasia
Diamond-Blackfan syndrome
Acquired pure red cell aplasia
Infection/Inflammation
(ineffective EPO production)
Medications
Bone marrow damage
Coombs Negative
Acute blood loss
Microangiopathic haemolytic anemia
Membrane defects
Hemoglobinopathies
Enzyme defects
Coombs Positive
Autoimmune hemolytic anemi
MCV 80-100 fL, MCH >27 pg
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Reticulocyte Count
Reticular [mesh-like] network of ribosomal RNA
Fully mature within 2 days (maturation)
Low reticulocyte <2.5%: Decreased marrow production of RBC
High reticulocyte >2.5%: Increased destruction of RBC
Immature RBC, normal 1%
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Anemia
แนวทางการจยและกษาภาวะโลตจาง
1. นกการจยของแพท
2. ประและการตรวจางกาย
3. ผลตรวจทางองปการ
4. การกษา หอการตรวจเมเม
การสปเวชระเยนและการใรส ไสามารถสปและใรสชดของภาวะโลตจาง
โดยจารณาจากผลการตรวจเอด Complete Blood Count เาน
โดยไการนกำจยในเวชระเยนของแพท
an=absence, hemia=blood, anemia=without blood
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Anemia from Acute Blood Loss
เกณการจย
1. ประการเยเอดอางเยบพนดเจน
2. ผลตรวจเอดสบสนาด
3. หอไการใเอดไ
4. นกการจยของแพท
“Acute post-hemorrhagic anemia”
“Anemia from acute blood loss”
“Acute blood loss”
“Anemia from blood loss”
Acute post-hemorrhagic anemia
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วยอาเยนเนเอด องกองกระเพาะอาหารพบ acute
hemorrhagic gastritis ผลตรวจ Hct เน 35% และใ PRC 2 ต
PDx Acute hemorrhagic gastitis K29.0
SDx
Anemia from acute blood loss D62
Op
Esophagogastroduodenoscope
Pack red cell transfusion
45.13
99.04
EC
12
วยรถมอเตอไซคำ ญญาณพปก รอยำชายโครงางาย เอกซเรคอมวเตอององพบ subcapsular
hematoma of spleen with hemoperitoneum เจาะเอดดตาม
Hct ลดลงจาก 40% เน 35%
PDx Splenic injury S36.00
SDx
Acute post-hemorrhagic anemia D62
Op
CT abdomen 88.01
EC มอเตอไซคำเอง V28.4
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Anemia Posthemorrhagic Anemia
ประการเยเอดอางเยบพนดเจน
ผลตรวจเอดพบ normochromic normocytic, MCV >80 fL
นกการจยของแพท
การกษาหอไการกษาโดยการใเอดไ
D62 Acute posthaemorrhagic anaemia Excludes: congenital anaemia from fetal blood loss ( P61.3 )
D63* Anaemia in chronic diseases classified elsewhere
œ
Anemia from acute blood loss
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Iron Deficiency Anemia
Common nutritional deficiency
Bleeding is a leading cause of iron deficiency anemia
Microcytic hypochromic anemia [low MCV, MCH & MCHC]
Iron studies
- Low serum iron
- High total iron binding capacity (TIBC, transferrin conc.]
- Low % transferrin saturation
- Low ferritin
- Decrease BM storage iron (hemosiderin)
Hypochromic anemia
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Iron Deficiency Anemia
ผลตรวจเอดพบ hypochromic microcytic, MCV <80 fL,
anisocytosis & poikilocytosis ไมาก
อาจสบสนวย serum iron ำ, TIBC ง, iron storage ำ
การกษาโดยการใเหกทดแทน
กรประการญเยเอดดเจน
ในกา Iron deficiency anemia due to chronic blood loss
กรไประการญเยเอด
ในกา Iron deficiency anemia
Hypochromic anemia
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Anemia in Chronic Disease
IFN, TNF, IL block iron transfer from macrophage store to RBC
Mild anemia resistant to iron therapy
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Anemia in Chronic Disease
Mild to moderate anemia
Mildly microcytic or Normocytic anemia
Etiology – Chronic immune activation
Chronic infections: TB, SBE, Osteomyelitis
Collagen vascular disease: RA, SLE
Endocrine deficiency: Hypothyroidism, Panhypopituitarism,
Hypoadrenalism
Visceral organ failure: Renal failure, Hepatic failure
Malignancy: BM metastasis, Leukemia, Lymphoma, Myeloma
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Anemia in Chronic Disease
โรคเองำใเดภาวะโลตจาง
✦ Chronic kidney disease stage 3–5
✦ Endocrine disease: Hypothyroidism [not…DM, DLP]
✦ Connective tissue disease: Rheumatoid
✦ Chronic infection: Tuberculosis, AIDs, Liver disease
ผลตรวจเอดพบ normochromic normocytic, MCV >80 fL
อาจนนวย serum ferritin ปก, TIBC ำ, iron storage ปก
การกษาวยการใเอด หอการกษาวย erythropoietin
หอการตรวจเมเม
Not record as Principle diagnosis
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Blood Loss from Procedure
เกณการจย
1. นกการเยเอดในรายงานการาด
2. ผลตรวจเอดสบสนาด
3. หอไการใเอดไ
4. นกการจยของแพท
“Operative bleeding”
“Postoperative bleeding”
“Postoperative anemia” Postoperative anemia = D64.9
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Anemia
Summary Diagnosis ICD-10
Iron deficiency anemia
Iron deficiency anemia due to chronic blood loss
Anemia from chronic blood loss
Anemia from blood loss
D50.9
D50.0
D50.0
D50.0
Anemia from acute blood loss
Acute posthemorrhagic anemia
D62
D62
Anemia in chronic disease
Anemia in neoplasm
D63.8*
D63.0*
Operative bleeding
Postoperative anemia due to acute blood loss
T81.0
D62
Most specific type as possible
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Anemia in Cancer Patient
Decreased Production Increased Destruction Blood Loss
Anemia of inflammation
Bone marrow infiltration
Chemotherapy
Chronic kidney disease
Nutritional deficiency
!
Hemolysis Acute blood loss
Chronic blood loss
Cancer-related anemia (CRA)
CRA (non-chemotherapy-related)
Chemotherapy-induced anemia
22
Anemia in Neoplastic Disease
มะเงงเนอ (active)
ผลตรวจเอดพบ normochromic normocytic, MCV >80 fL
อาจนนวย serum ferritin ปก
TIBC ปกหอ
serum/bone marrow ปกหอง
การกษาวยการใเอด หอการตรวจกษาเมเม
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Chemotherapy-Induced Anemia
Incidence increased from 19.5% in cycle #1 to 46.7% by cycle #5
Time to nadir hemoglobin level estimated at 2 weeks
Treatment: RBC, Erythropoietin stimulating agent (ESAs)
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Anemia in Cancer Patient
Summary Diagnosis ICD-10
Anemia in neoplastic disease
Chemotherapy-induced anemia (CIA)
Chemotherapy-induced aplastic anemia
D63.0*
D60.1
D61.1
Antimetabolites
Natural products
Antineoplastic antibiotics
Miscellaneous
Y43.1
Y43.2
Y43.3
Y43.3
Note: Chemotherapy-induced anemia, ICD-10-CM = D64.81
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Anemia in Cancer PatientSummary Diagnosis ICD-10
!
Folic acid analogue: Methotrexate
Pyrimidine analogue: 5-FU, Capecitabine, TS-ONE,
Cytarabine, Gemcitabine
Purine analogue: Mercaptopurine
Y43.1
!
Vinca alkaloids
Taxanes
Epipodophyllotoxins
Camptothecins: Irinotecan, Topotecan
Y43.2
!
Thalidomide, AzathioprineY43.4
Antineoplastic Antimetabolites
Antineoplastic Natural Products
Immunosuppressive Agents
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Anemia in Cancer PatientSummary Diagnosis ICD-10
other Antineoplastic Drugs
Antineoplastic antibiotics: Actinomycin, Doxorubicin,
Bleomycin, Mitomycin C, Mitomycin, Mitoxantrone,
Asparaginase
Alkylating agents: Chlorambucil, Cyclophosphamide,
Ifosfamide, Busulfan, Streptozocin, Meclorethamine,
Melphalan, Nitrourea, ThioTEPA
Miscellanous: Cisplatin, Carboplatin, Hydroxyurea
Target agents
Rituximab, Trastuzumab, Cetuximab, Bevacizumab,
Gemtuzumab
Y43.3
Note: จารณาตามกมของยา (อาจางจาก Table of Drugs And Chemical)
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Thalassemia
Inherited autosomal recessive blood disorder
characterised by abnormal formation of haemoglobin
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3 Basic Types of Hemoglobin
HbA : 2 alpha + 2 beta chains forming a tetramer
97% of adult haemoglobin
HbA2 : 2 alpha and 2 epsilon chains
1.5–3% of adult haemoglobin
HbF : 2 alpha and 2 delta chains
1% of adult haemoglobin
70–90% at term, fall to 25% by 1st month and progressively
First discovery in 1954
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Alpha ThalassemiaGenes delation on chromosome 16
2
Normally, there are 4 genes for alpha globin with 2 genes on each chromosome
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Alpha ThalassemiaGenes delation on chromosome 16
Silent carrier [3 functional alpha globin genes]
asymptomatic
Alpha Thalassemia Trait [2 functional alpha globin genes]
no anemia / mild anemia
microcytosis
Hemoglobin H disease [1 functional alpha globin gene]
microcytosis & hemolysis (results in severe anemia)
bone deformities (overcompenasation by bone marrow)
splenomegaly (peripheral hemolysis)
Golf ball inclusions on microscopy
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Alpha ThalassemiaGenes delation on chromosome 16
Alpha Thalassemia Major [no functional alpha globin genes]
HB Bart’s [high oxygen affinity]
fatal hydrops fetalis [edema and ascites due to accumulation
serous fluid as result of severe anemia]
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Beta ThalassemiaGenes mutation on chromosome 11
Hundreds of mutations possible in the beta thalassemia, therefore
beta thalassemia is more diverse
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Beta ThalassemiaGenes mutation on chromosome 16
Silent carrier
Beta Thalassemia Minor (Trait)
asymptomatic
microcytosis, minor anemia [Hb >10 g/dL]
Beta Thalassemia Intermedia
less severe to Cooley anemia [Hb 8–10 g/dL]
Beta Thalassemia Major (Cooley anemia)
moderate to severe anemia [Hb <7mg/dL]
present younger than 2 years old
bone deformities (overcompenasation by bone marrow)
splenomegaly (peripheral hemolysis)
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Thalassemia
เกณการจย
1. ภาวะเอดจางชด microcytic anemia; MCV <80 fL
2. กษณะ hypochromic, anisocytosis, poikilocytosis
3. อาการทางคก: thalassemia facies, hepatosplenomegaly
4. อาจระบ serum ferritin ปกหอง
5. อาจผลตรวจ hemoglobin electrophoresis
ไองนกและใรสจย
: D64.9 Anemia, unspecified
: D63.8 Anemia in other chronic disease classified elsewhere
‘Thalassa' = Great Sea (Mediterranean sea)
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Thalassemia
Summary Diagnosis ICD-10
Alpha thalassemia [Alpha thalassemia major]
Hemoglobin H disease [Alpha thalassemia intermedia]
Hemoglobin constant spring
D56.0
Beta thassemia [Beta thalassemia major]
Beta thalassemia / Hemoglobin E
Beta thalassemia intermedia
D56.1
Alpha thalassemia trait [Alpha thalassemia minor]
Beta thalassemia trait [Beta thalassemia minor]
Thalassemia trait [Thalassemia minor]
D56.3
Homozygous hemoglobin E
Hemoglobon E traitD58.2
Thalassemia D56.9
‘Thalassa' = Great Sea (Mediterranean sea)
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Thalassemia Complications
Transfusion-Transmitted Infections
Transfusion Iron Overload
Toxicities of Iron Chelation Therapy
Bacterial Infections
Cardiac Complications
Liver Complications
Bone Complications
Growth Retardation
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Iron Overload
Each unit of blood has 200 mg of iron
Iron loading rate depends upon number of units transfused
!
!
!
!
!
Hemosiderosis
: accumulation of hemosiderin, no significant tissue destruction
Hemochromatosis
: directly deposited in the tissue, may be genetic or acquired
GroupDaily Intake
mg/kg/d
Annual Intake of PRC
ml/kg
Low <0.3 <150
Moderate 0.3 - 0.5 150 - 250
High >0.5 >250
Hemosiderosis, Hemochromatosis, Iron poisoning
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Iron Overload
Iron poisoning
: acute overdose, may cause severe or even fatal symptoms due
to toxic effect of free iron in plasma which may be life threatening
: nausea, vomiting, abdominal pain, diarrhea
Hemosiderosis, Hemochromatosis, Iron poisoning
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Acquired Hemochromatosis
Caused by excess intake of iron, increased hemolysis and
repeated blood transfusion
Related to the involved organ system
Secondary hemochromatosis
Liver: Cirrhosis
Pancreas: Diabetic mellitus
Skin: Pigmentation, Bronzed diabetes
Endocrine: Hypothyroidism, Testicular atrophy
Joints: Arthritis
Heart: Arrhythmia, Heart Failure
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Iron Overload
Summary Diagnosis ICD-10
Iron overload
Hemosiderosis
Hemochromatosis
Congenital hemochromatosis
Acquired hemochromatosis
Transfusion hemochromatosis
Transfusion hemosiderosis
Iron toxicities
R79.0
E83.1
E83.1
E83.1
E83.1
E83.1
T80.8
T45.4
Hepatic hemochromatosis
Diabetic hemochromatosis
Arthritis in hemochromatosis
Cardiac hemochromatosis
E83.1
E83.1
E83.1 + M14.5
E83.1
Hemosiderosis, Hemochromatosis, Iron poisoning
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Iron Chelation Therapy
Deferoxamine Deferiprone Deferasirox
Brand Name Deferal Ferriprox Exjade
Half-life 20 minutes 2-3 hours 8-16 hours
Route SQ, IV PO PO
Dose (mg/kg/d) 20-60 75-100 20-40
Frequency 5-7 days/week 3 times daily Once daily
Iron Excretion Urine/Stool Urine Stool
Side EffectsVision, Hearing, Growth,
Local Reactions, Allergy
GI Symptoms, Kidney
Dysfunction, Hepatitis
GI Symptoms,
Agranulocytosis/
Neutropenia. Arthralgia
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Autoimmune Hemolytic Anemia
Hemolytic Anemia
43
Hymolytic Anemia
Normal RBC life 110–120 days
Anemia result from an increase in RBC destruction coupled with
increased erythropoiesis
Reduced RBC life span
The course of the disease Acute Chronic
The place of destruction Intravascular Extravascular
The whence Acquired Hereditary
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Predominantly Intravascular Hemolysis Predominantly Extravascular Hemolysis
Extracorpuscular (agents form outside the RBC)
Immune hemolysis: cold antibody
Microangiopathic hemolysis
Infectious agents, as in malaria
Thermal injury
Chemicals/Drugs
Venoms
Acute hemolytic transfusion
Prosthetic heart valves
Immune hemolysis: warm antibody
Membrane abnormalities
Spur cell anemia of severe liver disease
Paroxysmal nocturnal hemoglobinuria
Membrane defects [spherocytosis]
Intracorpuscular (abnormalities of the RBC interior)
Enzyme defects [G6PD deficiency]
Globin abnormalities [sickle cell
blue = acute hemolysis red = chronic hemolysis
Acq
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Here
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Extr
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Hemolytic Anemia
Summary Diagnosis ICD-10
Autoimmune hemolytic anemia (AIH)
Warm AIH
Cold AIH
Drug-induced AIH
D59.1
D59.1
D59.1
D59.0
Hemolytic-uremic syndrome
HELLP syndrome
D59.3
O1.42
Microangiopathic hemolytic anemia
Mechanical hemolytic anemia
Transfusion hemolysis
D59.4
D59.4
T80.8
Extracorpuscular
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Hemolytic Anemia
Summary Diagnosis ICD-10
Paroxysmal nocturnal hemoglobinuria D59.5
Spherocytosis
Stomatocytosis
Elliptocytosis
Ovalocytosis
D58.0
D58.8
D58.1
D58.1
Membrane abnormalities
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Hymolytic AnemiaReduced RBC life span
Intravascular hemolysis Extravascular hemolysis
Microangiopathic hemolysis
Paroxysmal nocturnal hemoglobinuria
Infections
Toxins
Hypersplenism
Hemoglobinopathies
Enzymopathies
Membrane defects
Autoimmune hemolytic anemia
Polychromasia
Reticulocyte count: increased
Unconjugated bilirubin: increased
Plasma free hemoglobin: normal
Urine bilirubin: +
Urine hemosiderin: +
Urine hemoglobin: +
Polychromasia
Reticulocyte count: increased
Unconjugated bilirubin: increased
Plasma free hemoglobin: increased
Urine bilirubin: +
Urine hemosiderin: 0
Urine hemoglobin: 0
H=hereditary; A=acquired
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