HEMOLYTIC ANEMIAS Flashcards

1
Q

causes?

A

intrinsic

  • membrane defects
  • enzyme deficiencies
  • unstable hemoglobin

extrinsic

  • antibody mediated
  • traumatic/mechanical
  • Infection

intravascular hemolysis

extravascular hemolysis

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2
Q

hemoglobinemia, hemoglobinuria are examples of?

A

intravascular hemolysis, hemolytic anemias

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3
Q

results in release of free hemoglobin?

A

hemoglobinemia

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4
Q

free hemoglobin excreted in the urine?

A

hemoglobinuria

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5
Q

this occurs within the reticuloendothelial system, like in the spleen?

A

extravascular hemolysis

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6
Q

what are the types of membrane defects?

A

hereditary spherocytosis

hereditary elliptocytosis

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7
Q

this is what type of membrane defect:

autosomal dominant
defect in cytoskeleton protein causing instability of membrane and formation of spherocytes
no definitive treatment, although splenectomy may be useful

A

hereditary spherocytosis

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8
Q

this is what type of membrane defect:

AD inheritance, 90% benign
cytoskeletal protein defects
altered erythrocyte shape, increased splenic clearance

A

hereditary elliptocytosis

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9
Q

what are the types of RBC enzyme deficiencies?

A

G6P dehydrogenase deficiency

other enzymatic deficiencies

  • pyruvate kinase
  • pyrimidine-5-nucleotides
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10
Q

most common RBC enzyme deficiency?

this is the first enzyme in?

A

G6P dehydrogenase deficiency

hexose monophosphate shunt, converting glutathione to reduced state, antioxidantst protection of RBC

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11
Q

accumulation of oxidized hemoglobin and other proteins results in?

A

Heinz bodies from iron deposits

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12
Q

patients have episodic anemia in times of oxidative stress due to?

A

infection
sulfa drugs
fava beans

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13
Q

this is necessary for glycolysis?

A

pyruvate kinase

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14
Q

this is necessary for RNA metabolism?

deficiency results in?

A

pyrimidine-5-nucleotidase

pyrimidine accumulation
basophilic stippling
hemolytic anemia

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