CH 18 Hemolytic Anemia - Enzyme Deficiencies

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7 Terms

1
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What are the 3 effects of G6PD deficiency?

NADPH generation impaired, GSH generation impaired, cellular oxidants accumulate

2
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What is the pathophysiology of G6PD deficiency?

G6PD deficiency → oxidation of Hgb → Heinz bodies → bite cells

3
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Consuming what specifically can cause G6PD deficiency?

fava beans

4
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What are the lab tests used to detect/measure G6PD?

Qualitative Fluorescent Spot test: Fluorescence of added G6P indicated G6PD deficiency

Quantitative spectrophotometric test: measures rate of NADP → NADPH

5
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What are the 3 pathophysiologic effects of pyruvate kinase deficiency?

decreased ATP production →

  1. cell membrane damage

  2. failure of cation pump

  3. cell dehydration


6
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What is the primary abnormal RBC morphology observed in pyruvate kinase deficiency?

Burr Cells

7
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What pathways does G6PD deficiency & PK deficiency affect?

G6PD: Hexose Monophosphate Shunt

PK: EMP