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

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

25
what causes acute cold autoimmune hemolytic anemias against big I
mycoplasma peumoniae
26
what causes acute cold autoimmune hemolytic anemias against big i
infectious mononucleosis
27
Paroxysmal cold hemoglobinuria is an autantibody against what
IgG (donath-Landsteiner antibody)
28
Paroxysmal cold hemoglobinuria what happens at low temp and warm temp
low: no hemolysis warm: binds complement
29
a patient went sking and has dark urine
paroxysmal cold hemoglobinuria
30
what is the difference between Paroxysmal cold hemoglobinuria and CAD
biphasic
31
how do you test for hemolysis
Coombs test
32
another name for hemolytic disease of newborn
erythroblastosis featlis
33
why does hemolytic disease of newborn occur
maternal antibodies
34
microangiopathic hemolytic anemias are due to
abnormalities in microcirculation
35
what is seen in blood smear for late stanges of alcohlic liver disease
spur cells | target cells
36
Paroxysmal nocturnal hemoglobinuria is a mutation of what
pig-A/PIGA
37
in Paroxysmal nocturnal hemoglobinuria what does PIg-A synthesize
GPI anchor | CD 59, 55 (complement)
38
CD 59
MIRL
39
CD 55
DAF
40
Paroxysmal nocturnal hemoglobinuria is senstive to what system to lysis
complement
41
how do you diagnose Paroxysmal nocturnal hemoglobinuria
flow cytometry
42
artifical heart valves can lead to what
iron deficiency
43
Reticulocyte counts differentiate between what
hemolysis and marrow failure
44
gel electrophoresis and HPLC detect what
abnormal hemoglobins
45
what does Coombs test detect
autimmune hemolytic anemias
46
plasma unconjugated bilirubin and haptoglobin concentrations support what diagnosis
hemolytic anemia
47
serum and red cell folate, vit B12 concentrations determine what
cause of megalobastic anemia