Hemolytic Anemia due to MEMBRANE DISORDERS

Question 1

• Inherited as a non-sex-linked dominant trait
• Most common in North Europeans
• Spherocytes die prematurely
• Splenomegaly

A. Hereditary spherocytosis/ Congenital Hemolytic Jaundice Anemia
B. Paroxysmal Nocturnal Hemoglobinuria (PNH) /Machiafava-Micheli Syndrome

Answer 1

A. Hereditary spherocytosis

Question 2

• Chronic intravascular hemolysis
• Nocturnal hemoglobinuria occurs during sleep or after
  awakening
• pH of plasma is low due to red cells
• Acidosis → Depressed respiration while sleeping
  (retention of Carbon dioxide resulting to acidosis)

A. Hereditary spherocytosis/ Congenital Hemolytic Jaundice Anemia
B. Paroxysmal Nocturnal Hemoglobinuria (PNH) /Machiafava-Micheli Syndrome

Answer 2

B. Paroxysmal

Question 3

Laboratory Findings:
✓ Positive sucrose hemolysis test or Ham’s acidified
serum test or sugar water test
✓ WBC count and platelet count are often low
✓ Hemosiderinuria is a feature

A. Hereditary spherocytosis/ Congenital Hemolytic Jaundice Anemia
B. Proxymal

Answer 3

B. Proxymal

Question 4

Peripheral smears:
✓ Blood film shows microspherocytes

A. Hereditary spherocytosis/ Congenital Hemolytic Jaundice Anemia
B.paroxymal

Answer 4

A. Hereditary

Question 5

-Inherited as a dominant characteristic associated with severe hemolytic anemia in infants
- Defect involves the impaired association of spectrin dimers resulting in free, unconnected dimers

A. Hereditary Elliptocytosis/Ovalocytosis
B. Hereditary Pyropoikilocytosis

Answer 5

A. Hereditary elliptocytosis

Question 6

Laboratory findings:
✓ Osmotic fragility test is increased
✓ Autohemolysis of red cells is present

Peripheral smears:
✓ Non-hypochromic elliptocytes are abundant on blood films
✓ Reticulocytes and NRBC are normal in shape

Answer 6

A. Hereditary elliptocytosis

Question 7

Rare moderately severe congenital hemolytic anemia
Inherited as recessive autosomal traits Occurs in black individuals/people

Peripheral smears:
✓ Microcytosis, striking micropoikilocytosis and fragmentation

A. Hereditary Stomatocytosis (Hydrocytosis)
B. Hereditary Pyropoikilocytosis
C. Hereditary Acanthocytosis (Abetalipoproteinemia)

Answer 7

B. Pyropoikilocytosis

Question 8

Rare congenital anemia
- Inherited as recessive autosomal trait
caused by increased Sodium and decreased Potassium due to increased permeability of membrane
- 10-30% red cells appear as mouth like

A. Hereditary Stomatocytosis (Hydrocytosis)
B. Hereditary Pyropoikilocytosis
C. Hereditary Acanthocytosis (Abetalipoproteinemia)

Answer 8

A. Stomatocytosis

Question 9

• Associated with plasma lipid abnormalities
• Low total lipid, cholesterol & phospholipids
• Autohemolysis occurs
• Reticulocyte count ranges from normal to
  increased
• Presence of mild anemia
  - Usual anticoagulant used: EDTA

A. Hereditary Stomatocytosis (Hydrocytosis)
B. Rh null
C. Hereditary Acanthocytosis (Abetalipoproteinemia)

Answer 9

C. Hereditary Acanthocytosis (Abetalipoproteinemia)

Question 10

• Inherited due to gene suppression or presence of silent Rh gene (X0)
• Membrane abnormalities due to absence of all Rh-Hr antigens on the red cells

A. Hereditary Stomatocytosis (Hydrocytosis)
B. Rh null
C. Hereditary Acanthocytosis (Abetalipoproteinemia)

Answer 10

B. Rh null

Question 11

Laboratory findings:
✓ High reticulocyte count
✓ Autohemolysis and osmotic fragility are increased

Peripheral smears:
✓ Mild, chronic normocytic normochromic hemolytic anemia
✓ Smear shows stomatocytes and spherocytes

A. Hereditary Stomatocytosis (Hydrocytosis)
B. Rh null
C. Hereditary Acanthocytosis (Abetalipoproteinemia)

Answer 11

B.rh null

Question 12

• Inherited
• Represents an imbalance in the
  membrane phospholipids in the red
  cells
• Anemia may increase due to infection
  or under condition of stress

A. Hereditary Stomatocytosis (Hydrocytosis)
B. Rh null
C. Hereditary Acanthocytosis (Abetalipoproteinemia)
D. High Phosphatidylcholine Hemolytic Anemia

Answer 12

D. High Phosphatidylcholine Hemolytic Anemia