Anemia part 3 Flashcards

1
Q

Intrinsic inherited hemolytic anemias: qualitative defects

A

sickle cell disease and HbC disease

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2
Q

what does the peripheral blood smear of a single RBC look like in hereditary spherocytosis

A

lack central palor

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3
Q

hereditary spherocytosis is a mutation in what? which produces what

A

RBC membrane skeleton protein gene, ankyrin

- anchor

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4
Q

what happens to hereditary spherocytosis when RBC pass through spleen

A

become spherocytes

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5
Q

hereditary spherocytosis deficiency is what

A

spectrin - membrane protien

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6
Q

how does one diagnose hereditary spherocytosis and are the results

A

osmotic fragility test

  • microcytosis
  • increase MCHC
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7
Q

hereditary spherocytosis treatment

A

folic acid supplements

- splenectomy

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8
Q

Glucose-6-phosphate dehydrogenase deficiency has a defective what

A

red cell enzyme

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9
Q

Glucose-6-phosphate dehydrogenase deficiency may protect from what disease

A

falciparum malaria

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10
Q

Glucose-6-phosphate do to the RBC

A

gives antioxidant protection

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11
Q

what happens to hemoglobin when there is oxidant stress without Glucose-6-phosphate dehydrogenase? what forms?

A

rbc denatured- forms Heinz body

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12
Q

what does Glucose-6-phosphate dehydrogenase make form NAD

A

NADPH

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13
Q

what is the role of NADPH

A

detoxify oxygen free radicals (hydrogen peroxide)

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14
Q

where are variants of Glucose-6-phosphate dehydrogenase deficiency located and severity

A

Mediterranean= severe

middle eastern

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15
Q

what are 3 precipitating factors for Glucose-6-phosphate dehydrogenase deficiency

A

infections
oxidant drugs ( sulfonamides, -quine, antimalarials)
fresh fava beans

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16
Q

is Glucose-6-phosphate dehydrogenase deficiency a constant occurrence in the body

A

no, acute attacks

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17
Q

what happens in autoimmune hemolytic anemias

A

autoantibodies attach to RBC

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18
Q

how is autoimmune hemolytic anemias detected

A

combs test

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19
Q

warm autoimmune hemolytic anemias react with what anitbody

A

IgG

20
Q

what does the spleen do in warm autoimmune hemolytic anemias

A

eliminates IgG coated cells: marcophage Fc receptors bind to Fc ends of IgG antibodies

21
Q

what drugs can induce warm autoimmune hemolytic anemias

A

methyldopa
penicillin-hapten
tetracyclines
cephalosporins

22
Q

what disorder are associated with warmautoimmune hemolytic anemias

A

lymphatic malignancies

autoimmune disorders

23
Q

treatment for autoimmune hemolytic anemias

A

corticosteroids

24
Q

cold autoimmune hemolytic anemias target what antibody

A

IgM

25
Q

what causes acute cold autoimmune hemolytic anemias against big I

A

mycoplasma peumoniae

26
Q

what causes acute cold autoimmune hemolytic anemias against big i

A

infectious mononucleosis

27
Q

Paroxysmal cold hemoglobinuria is an autantibody against what

A

IgG (donath-Landsteiner antibody)

28
Q

Paroxysmal cold hemoglobinuria what happens at low temp and warm temp

A

low: no hemolysis
warm: binds complement

29
Q

a patient went sking and has dark urine

A

paroxysmal cold hemoglobinuria

30
Q

what is the difference between Paroxysmal cold hemoglobinuria and CAD

A

biphasic

31
Q

how do you test for hemolysis

A

Coombs test

32
Q

another name for hemolytic disease of newborn

A

erythroblastosis featlis

33
Q

why does hemolytic disease of newborn occur

A

maternal antibodies

34
Q

microangiopathic hemolytic anemias are due to

A

abnormalities in microcirculation

35
Q

what is seen in blood smear for late stanges of alcohlic liver disease

A

spur cells

target cells

36
Q

Paroxysmal nocturnal hemoglobinuria is a mutation of what

A

pig-A/PIGA

37
Q

in Paroxysmal nocturnal hemoglobinuria what does PIg-A synthesize

A

GPI anchor

CD 59, 55 (complement)

38
Q

CD 59

A

MIRL

39
Q

CD 55

A

DAF

40
Q

Paroxysmal nocturnal hemoglobinuria is senstive to what system to lysis

A

complement

41
Q

how do you diagnose Paroxysmal nocturnal hemoglobinuria

A

flow cytometry

42
Q

artifical heart valves can lead to what

A

iron deficiency

43
Q

Reticulocyte counts differentiate between what

A

hemolysis and marrow failure

44
Q

gel electrophoresis and HPLC detect what

A

abnormal hemoglobins

45
Q

what does Coombs test detect

A

autimmune hemolytic anemias

46
Q

plasma unconjugated bilirubin and haptoglobin concentrations support what diagnosis

A

hemolytic anemia

47
Q

serum and red cell folate, vit B12 concentrations determine what

A

cause of megalobastic anemia