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Heme > Hemolytic Anemia > Flashcards

Hemolytic Anemia Flashcards

1
Q

What is Intrinsic hemolytic anemia?

A

Disorder with the RBC itself like disorders of the red cell membrane, metabolic hemolysis, and disorders of Hb synthesis

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2
Q

What are examples of Intrinsic anemias involving disorders with the red cell membrane?

A

hereditary spherocytosis, hereditary elliptocytosis, stromatocytosis, acanthocytosis, and PNH

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3
Q

What are examples of Intrinsic anemias involving disorders of metabolic hemolysis?

A

Hexosemonophosphate shunt (G6PD) and pyruvate kinase disorders

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4
Q

What are examples of Intrinsic anemias involving disorders of Hb synthesis?

A

Sickling hemoglobinopathies and thalassemias.

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5
Q

What is extrinsic hemolysis?

A

Disorders that affect the RBC like Microangiopathic conditions, immune hemolytic anemia, infectious conditions, and hypersplenism.

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6
Q

What are examples of Microangiopathic conditions?

A

Cardiac valve mechanical hemolysis, TTP, DIC, and burns

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7
Q

What are some examples of immune hemolytic anemia?

A

Auto-immune hemolytic anemia(idiopathic and secondary), warm antibodies, cold antibodies, allo-immune hemolytic anemias, transfusion reactions, erthroblastosis fetalis

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8
Q

What are some examples of infectious conditions causing hemolytic anemia?

A

Malaria, babesiosis, and clostridial sepsis

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9
Q

What is the mechanism of extravascular hemolysis?

A

RBC phagocytosed by macrophages in the spleen and liver (RE system)

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10
Q

Why are RBCs phagocytosed in extravascular hemolysis?

A

RBCs are coated with IgG or and or C3b, or RBCs are abnormally shaped like sperocytes or sickle cells

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11
Q

Would you see an increase in unconjugated bilirubin in extravascular hemolysis?

A

Yes

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12
Q

Would you see an increase in LDH in extravascular hemolysis?

A

Yes

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13
Q

What is intravascular hemolysis?

A

Hemolysis that occurs within blood vessels caused by things like enzyme deficiencies, complement mediated destruction of RBCs, and mechanical damage (i.e. valve stenosis)

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14
Q

What two findings will you see in intravascular hemolysis?

A

Decreases serum haptoglobin and hemoglobinuria

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15
Q

What are the clinical features of hemolytic anemia?

A

Pallor of mucus membranes, jaundice, and splenomegally. There is No bilirubin in urine. May see gallstones (from bilirubin) and ulcers around the ankle (esp. sickle cell pts)

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16
Q

What characterizes Aplastic crises?

A

Parvovirus infection which usually turns off erythropoiesis, so you see a sudden drop in anemia and a drop in the reticulocyte count

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17
Q

What is hereditary spherocytosis?

A

Autosomal dominant disorder affecting mostly northern europeans. Mutation in ankyrin (most common) or spectrin, or band 3, which are all RBC membrane proteins

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18
Q

What are the clinical findings of hereditary sperocytosis?

A

Jaundice, gallstones, splenomegally, and aplastic crisis.

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19
Q

What are the lab findings of hereditary sperocytosis?

A

Normocytic anemia with sperocytes, increased MCHC, and increased osmotic fragility

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20
Q

How would you treat hereditary spherocytosis?

A

Splenectomy

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21
Q

What is hereditary elliptocytosis?

A

Autosomal dominant, defective spectrin.

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22
Q

What are the clinical findings of hereditary elliptocytosis?

A

Most have none to mild anemia and splenomegally

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23
Q

What are the lab findings hereditary elliptocytosis?

A

Elliptocytes on peripheral blood smear.

24
Q

What is the treatment of hereditary elliptocytosis?

A

Splenectomy only if symptomatic

25
Q

What is PNH?

A

Rare, acquired disorder in marrow stem cells. Absent DAF and CD59; therefore RBCs are no longer protected from complement deposition which results in intravascular hemolysis

26
Q

What are the clinical findings in PNH?

A

Hemoglobinuria which may cause Fe deficiency, thrombosis, and risk for developing acute myelogenous leukemia

27
Q

What are the peripheral blood findings in PNH?

A

Normocytic anemia with pancytopenia.

28
Q

How do you diagnose PNH?

A

Flow cytometry, sugar water test

29
Q

What is G6PD deficiency?

A

X linked recessive (affects males). Seen in mediterranean and blacks. G6PD is only source of NADPH, which is needed for glutathione. Deficiency causes RBCs to be susceptible to oxidant stress

30
Q

What are some oxidant stresses that induce hemolysis in G6PD deficiency?

A

Infection, drugs, and FAVA beans

31
Q

What are the lab findings in G6PD deficient pts?

A

Normocytic anemia, heinz bodies, and bite cells

32
Q

What are the clinical findings of G6PD deficient pts?

A

Sudden onset of back pain with hemoglobinuria that occurs 2-3 d after oxidant stress

33
Q

How do you diagnose G6PD deficiency?

A

Enzyme level test. However, this can only be done when pt is not in active hemolysis, otherwise risk test giving a false “normal” level

34
Q

What is pyruvate kinase deficiency?

A

Autosomal recessive. Chronic lack of ATP causing membrane rigid.

35
Q

What are the clinical findings of pyruvate kinase deficiency?

A

Anemia with jaundice and gallstones. Increase in 2,3 DPG synthesis causes right shift oxygen dissociation curve (this somewhat offsets the effects of anemia)

36
Q

What are the lab findings of pyruvate kinase deficiency?

A

Normocytic anemia and echinocytes (RBCs with thorny projections)

37
Q

What is the most common cause of immune hemolytic anemia?

A

Autoimmune warm type (IgG)

38
Q

What immunoglobulin is found with cold autoimmune hemolytic anemia?

A

IgM

39
Q

What is the pathogenesis of warm (IgG) mediated hemolysis?

A

RBCs coated with IgG are phagocytosed by splenic macrophages, typical of extravascular hemolysis and spherocytes are produced if some of the membrane is removed

40
Q

What are the clinical findings of autoimmune hemolytic anemias?

A

Jaundice (extravasular), splenomegally, raynauds phenomena (cold types)

41
Q

What are the lab findings in warm autoimmune hemolytic anemia?

A

Spherocytosis, positive direct Coombs( DAT)

42
Q

What is the treatment for warm autoimmune hemolytic anemia?

A

Remove the underlying cause (i.e. drug induced), give corticosteriods, splenectomy if don’t respond well to other treatments, immunosuppressives if don’t respond to corticosteriods, mAbs, or IV Ig’s

43
Q

What is the pathogenesis of cold autoimmune hemolytic anemia?

A

IgM mediated hemolysis that fixes complement. Can be either intravascular (more common) or extravascular

44
Q

What are the lab findings of cold autoimmune hemolytic anemia?

A

SImilar to warm, except red cells agglutinate in cold

45
Q

What is the treatment of cold autoimmune hemolytic anemia?

A

Keep patients warm and treat any underlying cause. Can give rituximab or anti-CD52 mAbs

46
Q

What are alloimmune hemolytic anemias?

A

Antibodies produced by one individual reacts with RBCs of another. Examples are ABO incompatibility and Rh disease of the newborn

47
Q

What are three mechanisms of drug induced hemolytic anemias?

A
  1. Ab directed against a drug-RBC (i.e. penicillin)
  2. Deposition of complement via drug-Ab complex
  3. True autoimmune hemolytic anemia with unclear role of the drug
48
Q

What are red cell fragmentation syndromes?

A

Arise through physical damage to RBCs, i.e. artificial heart valves or microangiopathic anemias

49
Q

What is March hemoglobinuria

A

Damgage to RBCs between small bones of the feet like in marathoners (aka Runners anemia)

50
Q

What are microangiopathic hemolytic anemias?

A

Microcirculatory lesions that cause RBC fragmentation; schistocytes

51
Q

What are macroangiopathic hemolytic anemias?

A

Hemolytic process caused by valvular defects (aortic stenosis)

52
Q

What are the lab findings in micro and macroangiopathic hemolytic anemias?

A

Normocytic anemia, long standing hemoglobinuria causes Fe deficiency. Decreases serum haptoglobin and hemoglobinuria, and schistocytes

53
Q

What is MCV?

A

hct/RBC*10

54
Q

What is MCH?

A

Hb/RBC*10

55
Q

What is MCHC?

A

Hb/Hct*100

56
Q

What tests do you look for in the evaluation of hemolysis?

A

Peripheral smear, retic count, bilirubin, LDH, plasma Hb, urine Hb, urine hemosiderin, haptoglobin, DAT, G6PD screen, Hams test, sugar water test, osmotic fragility

57
Q

Should malaria be considered as a potential cause of hemolytic anemia?

A

Yes

Heme (38 decks)

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