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Hem part 2 > 120914 hemolytic anemias cases > Flashcards

120914 hemolytic anemias cases Flashcards

1
Q

when RBCs are spherocytes, what happens to their MCHC?

A

increased compared to normal RBCs

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2
Q

when RBCs are spherocytes, what happens to their MCV?

A

normal compared to normal RBCs

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3
Q

conditions with spherocytes can be inherited or acquired?

A

both

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4
Q

osmotic fragility test

A

only identifies presence of spherocytes (but so does the peripheral blood smear)

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5
Q

what is IgG mediated immune hemolytic anemia associated w?

A

lupus (most common cause)
CLL
certain drugs

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6
Q

Coombs test positive indicates

A

immune hemolytic anemia

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7
Q

spherocytes are observed in

A

hereditary spherocytosis
disorders characterized by splenic bites (autoimmune hemolysis)

thermal injury

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8
Q

hereditary spherocytosis-why does it occur?

A

the cytoskeleton is weakened and the cell membrane is weakaned, causing blebs to come off

then once a spherocyte, can get engulfed by splenic macrophage

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9
Q

clinical findings for hereditary spherocytosis

A 
wide variability in severity of hemolysis
splenomegaly
aplastic crisis (parvovirus infects/kills erythroid precursors)
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10
Q

diagnosing hereditary spherocytosis–what do you look for?

A

family hx

peripheral blood review-see spherocytes-with negative direct antiglobulin test

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11
Q

warm autoantibody type autoimmune hemolytic anemia is caused by?

A

idiopathic (60%)

secondary causes:
lymphoproliferative disorders like CLL, lupus, etc

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12
Q

cold autoantibody type AIHA is caused by?

A

idiopathic

secondary causes: postinfectious with mono or mycoplasma, lymphoproliferative disorders

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13
Q

cold agglutinins are seen in

A

cold autoantibody type AIHA

usually do not cause anemia, but may cause obstructive symptoms

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14
Q

clinical findings for warm AIHA

A

symptoms of anemia

organomegaly

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15
Q

cold AIHA clinical findings

A 
acute or chronic form
follows cold exposure
acrocyanosis
Raynaud phenomenon
can cause mild to severe anemia
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16
Q

Heinz body

A

precipitated hemoglobin (caused by oxidative stress)

17
Q

bite cells are produced by what mechanism?

A

macrophages phagocytosing denatured protein

18
Q

G6PD deficiency is acquired or inherited?

A

inherited- X linked recessive disorder

19
Q

why is splenectomy not good for G6PD deficiency pts?

A

b/c G6PD has hemolysis in intravascular and extravascular components

20
Q

glucose 6 phosphate dehydrogenase is functioning in which pathway?

A

pentose phosphate pathway

21
Q

glucose 6 phosphate dehydrogenase deficiency has what effects?

A

reduced levels of NADPH, which results in reduced glutathione, which results in inability to reduce oxidant stressors

22
Q

pathogenesis of G6PD deficiency

A

denatured Hb (Heinz bodies) directly damage the RBC, causing intravascular hemolysis

Heinz bodies are removed in the spleen, causing extravascular hemolysis

23
Q

oxidant stressors in the case of G6PD deficiency

A

infection
fava beans
drugs: dapsone, sulfonamides, primaquine, nitrofuantoin, quinolones

24
Q

G6PD deficiency is common in

A

malarial endemic areas

25
Q

G6PD deficiency occurs how

A

results in reduced half life of G6PD, so older RBCs are vulnerable to oxidative stress and subject to lysis

26
Q

what variant of G6PD deficiency is in 11% of African Americans

A

G6PD A-

27
Q

bite cells

A

G6PD deficiency

28
Q

how can you get mechanical trauma to RBCs?

A

extreme burn
defective cardiac valves
microangiopathic hemolytic anemias (TTP a MEDICAL EMERGENCY, malignant HTN, antiphosphlipid antibody syndrome, DIC, disseminated cancer)

29
Q

microangiopathy

A

disease of small blood vessel

30
Q

kidney failure, neuro symptoms, think?

A

thrombotic thrombocytopenic purpura

31
Q

paroxysmal nocturnal hemoglobinuria

A

rare disorder
ACQUIRED mutation in PIGA gene on X chromosome
results in decreased GPI-linked proteins b/c GPI is absent. lack of GPI-linked anti-complement proteins makes cells susceptible to complement lysis

32
Q

PNH affects what cells

A

stem cell disorder of all myeloid lineages, though RBCs are most sensitive to complement lysis

33
Q

schistocytes

A

microangiopathic hemolytic anemias

34
Q

DAT differentiates what from what?

A

spherocytosis disorders–differentiates hereditary spherocytosis from autoimmune hemolysis

35
Q

how can you diagnose PNH?

A

flow cytometry for GPI-linked proteins, looking at WBCs AND RBCs:
CD55(DAF)
CD59

36
Q

ex of red cell fragmentation disorder

A

PNH

Hem part 2 (11 decks)

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