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Blood > Hemolytic Anemia 9/27 > Flashcards

Hemolytic Anemia 9/27 Flashcards

1
Q

What molecule does free Hgb bind to in intravascular hemolysis?

A

Free Hgb binds to HAPTOGLOBIN

When binding capacity exceeds, plasma turns PINK

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2
Q

How do you determine intravascular hemolysis? 3 indications.

A
  1. Reduced serum haptoglobin
  2. Presence of plasma (pink) or urine (red dipstick) hemoglobin
  3. Detection of hemosiderin in renal tubular cells in urinary sediment
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3
Q

What are the 3 disorders intrinsic to RBCs that cause hemolytic anemia?

A
  1. Abnormal Hbg
  2. Enzyme defect: G6PD deficiency
  3. Membrane abnormality: spherocytosis
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4
Q

What are the 5 disorders extrinsic to RBCs that cause hemolytic anemia?

A
  1. Autoimmune
  2. Mechanical factors (burns)
  3. Infections & toxins (snake venom)
  4. Liver disease
  5. Hypersplenism
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5
Q

What should you test to determine hemolytic anemia? 5 tests

A
  1. CBC
  2. Blood smears
  3. Reticulocyte count
  4. EPO
  5. BM biopsy
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6
Q

What test is used to distinguish immune from non-immune mediated hemolysis?

A

Coomb’s antiglobulin test

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7
Q

How does Coombs test work?

A

Detects Ag/Ab complexes on RBC membrane.

Coombs reagents are antibodies that recognize the Fc portion of Ig or C3d.

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8
Q

What are features of hemolytic anemia diagnosis?

A
  1. Compensatory production of Reticulocytes
  2. Reticulocyte index >3
  3. Absolute count of rtcytes >100k
  4. Bilirubin elevated
  5. LDH elevated
  6. Serum haptoglobin diminished
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9
Q

what 2 tests are performed to diagnose extra vascular hemolytic anemia?

A
  1. Coombs

2. Hgb electrophoresis

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10
Q

What 2 tests determine intravascular hemolytic anemia?

A
  1. Hgb in urine and plasma

2. Iron stains of urinary sediment

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11
Q

What are the 3 heriditary/intrinsic erythrocyte defects of hemolytic anemia?

A
  1. Hereditary spherocytosis
  2. Heriditary elliptocytosis
  3. G6PD deficiency
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12
Q

How is hereditary spherocytosis inherited and what is the characteristic?

A

Autosomal.

Intrinsic defects in RBC membrane - SPHEROCYTES.

Reduced membrane stability.

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13
Q

Which erythrocyte membrane proteins lead to heriditary spherocytosis when defective? (4)

A
  1. Ankyrin
  2. Spectrin (most common)
  3. Band 3
  4. Band 4.2
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14
Q

What are symptoms of spherocytosis? (3)

A
  1. Anemia
  2. Splenomegaly
  3. Jaundice
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15
Q

How do you diagnose hereditary spherocytosis? (2)

A
  1. Osmotic fragility test

2. Molecular studies to confirm

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16
Q

What is the pathogenesis of g6dp deficiency?

A
  1. O2 oxidation
  2. Denaturation of globin chains
  3. Heinz bodies
  4. Cell rigidity
  5. Hemolysis

Blood (13 decks)

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