Transcript PowerPoint Presentation - Week 2: Hemolytic Anemia

Week 2: Hemolytic Anemia
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Normocytic anemia
Fanconi anemia
Diamond-Blackfan
(PRCA)
Types of hemolysis
Evidence of hemolysis
Reticulocyte
G6PD deficiency
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Heinz bodies
PK deficiency
Cytoskeleton
Spherocytosis
Elliptocytosis
PNH
Normocytic Anemia
Acute hemorrhage
 Hypo-proliferative
 Acquired aplastic anemia
 Constitutional aplastic anemia
 Pure red cell aplasia (PRCA)
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Acute Hemorrhage
No changes in CBC until plasma volume
increases
 Later, reticulocytosis
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Hypoproliferative Anemia
Pancytopenia
 < 25% BM cellularity
 Low retic count
 Bone marrow defect
 Immunologic suppression
 Growth Factor deficiency
 Abnormal stem cells
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Acquired Aplastic Anemia
Idiopathic
 Drugs and chemicals (eg, chloramphenicol,
benzene, insecticides)
 Radiation
 Virus
 Paroxysmal nocturnal hemoglobinuria
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Constitutional Aplastic Anemia
Fanconi’s anemia
 Chromosomal breaks
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Pure Red Cell Aplasia PRCA
Infections
 Drugs
 Diamond-Blackfan syndrome
 No EPO deficiency
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Hemolytic Anemia
Intravascular vs extravascular
 Bilirubin metabolism
 Types
 Membrane defect
 Metabolic defect
 Extracorpuscular
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Evidence of Hemolysis
Low RBC survival with chromium tagging
study
 Unconjugated bilirubin
 Plasma Hb
 Decreased serum haptoglobin
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Evidence of Erythropoiesis
Polychromasia
 Increased reticulocyte
 “Shift” macrocytosis
 Hypercelluar BM
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Correcting Retic Count
Retic Index = Retic % x
Patient Hct
Normal Hct
Absolute Retic = Retic % x RBC/mm3
Retic Production Index =
Retic Index
Days in circulation
Membrane Defect
Spherocytosis
 Elliptocytosis
 PNH (sensitivity to complement lysis -sugar water test, Ham’s test)
 Stomatocytosis (possibly Rh null)
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Spherocytes
Elliptocytes
Osmotic Fragility
Paroxysmal Nocturnal Hemoglobinuria
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Hematopoietic stem cell disorder
Mutation of phosphatidylinositol glycan class A
(PIG-A) gene
Glycosylphosphatidylinositol (GPI) anchors
membrane proteins
Without GPI, unable to regulate completment
activities on membrane
Hemolysis is pH dependent
Thrombosis can occur
Lab Tests for PNH
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Acidified serum lysis test (Ham’s test): PNH cells
lyse due to complement activation in acidified
serm
Sugar water (sucrose hemolysis) test: RBCs
sensitive to complement will lyse in sucrose and
serum
Flow cytometry: lack of CD59 on RBCs, or lack
of CD59 or CD55 on granulocytes
Stomatocytes
Metabolic Defect
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G6PD deficiency
 Hexose
monophosphate shunt
 Most common RBC
enzyme defect, >50
variants
 X-linked
 Low glutathione due to
low NADPH
 Oxidative lysis, Heinz
bodies, spherocytic
 Primaquine, fava beans
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Pyruvate kinase deficiency
 Glycolysis
 Low RBC ATP level
 Non-spherocytic
B12 and folate deficiency
 Macrocytic
 HJ bodies
Hemoglobinopathies
 Poikilocytosis
 Abnormal Hb
Heinz body preparation with Crystal violet
Unstable hemoglobin
Extracorpuscular Factors
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Antibodies
 Autoimmune
 Isoimmune
Drugs, antibiotics
Fresh water
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Abnormal plasma
lipids
 Acanthocytosis
Venom
 Snake
 Spider
 Bee
Extracorpuscular Factors
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Trauma
 DIC
 Hemolytic uremic
syndrome (HUS)
 TTP
 Angiopathy
 Heat
 Heart valves
 “March”
hemoglobinuira
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Microorganisms
 Malaria
 Babesia
 Clostridium
 Gram negative
endotoxin
Schistocytes
Malaria