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Heme > Lec 4 Hemolytic Anemia > Flashcards

Lec 4 Hemolytic Anemia Flashcards

1
Q

What is normal mech of RBC destruction?

A

normal RBCs liver for 4 months; disposal carried out by spleen “extravascularly”

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2
Q

What 3 things is hemoglobin degraded into?

A
  • amino acids from globin
  • Fe
  • biliverdin
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3
Q

What happens to biliverdin?

A

reduced to bilirubin and released in blood where carried to liver as unconjugated bilirubin

in liver conjugated and excreted through bile

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4
Q

What happens to free hemoglobin released from splenic hemolysis?

A

bound to haptoglobin carrier protein

taken up in liver and degraded by hepatic phagocytes

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5
Q

What clinical features should you expect to see in patients with extravascular hemolysis regardless of etiology?

A
  • jaundice [from unconjugated bili if liver function normal]
  • splenomegaly from work hypertrophy
  • increased RBC production [high EPO]
  • increased reticulocyte count + possibly nucleated RBCs
  • erythroid hyperplasia
  • high LDH from hemolysis
  • decreased haptoglobin b/c its being cleared wtih hemoglobin
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6
Q

What kind of bilirubin will you see if extravascular hemolysis and liver disease?

A

both conjugated and unconjugated

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7
Q

What is a complication of higher load of excreted bilirubin?

A

bilirubin/pigment gallstones

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8
Q

What should you see in peripheral blood smear of patient with extravascular hemolysis?

A
  • polychromasia [larger bluer looking RBCs = reticulocytes]

- nucleated RBCs

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9
Q

What does presence of spherocytes indicate?

A

autoimmune hemolysis; hereditary spherocytosis

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10
Q

What does presence of schistocytes indicate?

A

microangiompathic hemolysis due to DIC, thrombotic thrombocytopenic purpura [TTP],

traumatic hemolysis –> prosthetic heart valve

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11
Q

What does presence of target cells indicate?

A 
HbC disease
asplenia
sickle cell
thalassemia
liver disease
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12
Q

What does presence of acanthocytes indicate?

A

acanthocytes = spiny spur cels
liver disease
spur cell anemia
abetalipoproteinemia

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13
Q

What are some clinical consequences of intravascular hemolysis?

A
  • hemoglobinemia
  • hemoglobinuria
  • hemosiderinuria
  • increased LDH
  • increased retic
  • jaundice
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14
Q

What is warm agglutination autoimmune anemia? What type of hemolysis?

A

warm = IgG mediated; seen in SLE, CLL, methyl-dopa

extravascular hemolysis

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15
Q

What is cold agglutination autoimmune anemia? What type of hemolysis?

A

cold = IgM; acute anemia triggered by cold; seen in CLL, mycoplasma pneumonia, mono

intravascular hemolysis

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16
Q

Do you see splenomegaly in intravascular hemolysis?

A

nope b/c the spleen isn’t doing the work

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17
Q

What does the Coombs test tell you?

A

presence of IgM or IgG antibody on surface of RBC

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18
Q

What is direct coombs test?

A

anti-Ig antibody aded to patients blood

if RBCs agglutinate –> tells you RBCs are coated wtih Ig

19
Q

What is indirect coombs test?

A

normal RBC added to patient’s serum –> add anti-Ig antibodies

if serum has anti-RBC surface Ig it will bind the RBCs; then when you add the anti-Ig it will aggglutinate

20
Q

What do you see on peripheral smear with warm immune hemolytic anemia?

A
  • spherocyte
  • polychromasia [reticulocytes]
  • nucleated RBCs
21
Q

What do you see on CBC in autoimmune anemia?

A

high reticulocyte count

MCV normal or high due to elevated retic count

22
Q

What type of autoimmune anemia associated with EBV?

A

IgM = cold autoimmune hemolytic anemia

23
Q

What kind of autoimmune anemia do you get when ABO incompatible blood is transfused?

A

intravascular hemolysis = IgM mediated = Cold

24
Q

What kind of autoimmune anemia do you get when Rh/minor antigen incompatible blood is transfused?

A

extravascular = IgG mediated

not as severe at from ABO incompatible

25
Q

What is hereditary spherocytosis?

A

defect in RBC cell membrane cytoskeletal proteins –> membrane may be lost in some areas; lose biconcave shape

26
Q

What is the most common inherited RBC membrane disorder?

A

hereditary spherocytosis

27
Q

How is hereditary spherocytosis transmitted?

A

autosomal dominant

28
Q

What labs can you use to test for hereditary spherocytosis?

A
  • positive osmotic fragility = less ability to increase cell volume
29
Q

What do you see on peripheral smear in hereditary spherocytosis?

A

spherocytes

30
Q

What is treatment for hereditary spherocytosis?

A

splenectomy = removes site of hemolysis

31
Q

What is spur cell anemia?

A

changes in RBC shape b/c membrane enlarged w/out change in cell volume due to increased cholesterol in RBC membrane

caused by liver disease

32
Q

What do you see on smear in spur cell anemia?

A

spur cells [acanthocytes]

target cells

33
Q

What do you see in paroxysmal nocturnal hemoglobinuria?

A

loss of CD55 / CD59 –> complement mediated lysis of RBCs = intravascular hemolysis

34
Q

What is pathogenesis of G6PD deficiency?

A

G6OD helps produce NADPH for RBC –> used for reduction of glutathione which is used for reduction of hydrogen peroxide –> increased susceptibility to oxidant stress

35
Q

How is G6P dehydrogenase deficiency inherited?

A

x linked recessive

36
Q

Why do you see heinz bodies in G6PD deficiency?

A

heinz bodies = precipitants of hemoglobin that have been damaged by oxidants

37
Q

What kind of hemolysis do you see in G6PD deficiency?

A

intravascular hemolysis from oxidant damage to RBC cell membrane

extravascular hemolysis

38
Q

What are some classic causes of oxidant stress leading to hemolytic anemia in patients with G6PD deficiency?

A
  • infection
  • alcohol
  • sulfa drugs
  • antimalarials
  • fava beans
39
Q

What do you see on smear with G6PD deficiency?

A

RBCs with Heinze bodies and bite cells

40
Q

Why might G6PD level may be paradoxically normal during an acute hemolytic episode?

A

b/c there are higher levels of G6PD enzyme in reticulocytes; during episode –> have increased reticulocyte production

41
Q

What is the pathogenesis of microangiopathic anemia?

A

traumatic fragmentation of RBCs as they pass through obstructed blood vessels

42
Q

What do you see on peripheral smear in patient wtih microangiopathic anemia?

A

fragmented RBCs = schistocytes

43
Q

What are two conditions classically associated wtih microangiopathic hemolysis?

A
  • disseminated intravascular coagulation [DIC]

- thrombotic thrombocytopenic purpura [TTP]

Heme (20 decks)

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