HEMOLYTIC ANEMIA DUE TO METABOLIC DISORDERS

Question 1

TYPE OF DISORDER

 **inherited sex-linkes
 Complex heterogenous disorder which is uniquitous
 Seen in enzymes deficient hemolytic anemia
 Type A, Type B & Favism
**
A. Pyruvate Kinase Deficiency
B. G6PD deficiency/ Glucose-6-phosphate Dehydrogenase
Deficiency

Answer 1

B. G6PD deficiency/ Glucose-6-phosphate Dehydrogenase
Deficiency

Question 2

Most common defect seen in
enzyme deficient hemolytic
anemia

A. Pyruvate Kinase Deficiency
B. G6PD deficiency/ Glucose-6-phosphate Dehydrogenase
Deficiency

Answer 2

B. G6PD deficiency/ Glucose-6-phosphate Dehydrogenase
Deficiency

Question 3

usually seen in black individuals in G-6PD DISORDER

A. TYPE A
B. TYP B

Answer 3

A. TYPE A

Question 4

patients are more susceptible to sever oxidants hemolysis compared to type A; frequent in Mediterranean race

A. TYPE A
B. TYP B
C. FAVISM

Answer 4

B. TYP B

Question 5

G6PD deficiency

most delicate, a life threatening hemolysis

A. TYPE A
B. TYP B
C. FAVISM

Answer 5

C. FAVISM

Question 6

G6PDD)

**Laboratory Tests:
 Methyl Violet or Crystal Violet Stains
 Dye Reduction Test
**

a. Pyruvate Kinase Deficiency
b. G6PDD)

Answer 6

b. G6PDD)

Question 7

The most common red cell enzyme deficiency involving the Embden- Meyerhof glycolytic pathway

a. Pyruvate kinase deficiency
b. Pyrimidine-5-Nucleotidase (PN) Deficiency
c. Glucose Phosphate Isomerase Deficienc
d. Triosephosphate Isomerase, Hexokinase and
Diphosphoglycerate Mutase Deficiency

Answer 7

a. Pyruvate kinase deficiency

Question 8

- PK converted to phosphophenolpyruvate
to pyruvate in the EMP with the production of ATP
- RBC loses its flexibility due to decrease ATP
- Mild to moderately hemolytic anemia with
splenomegaly

.A. Pyruvate kinase deficiency
b. Pyrimidine-5-Nucleotidase (PN) Deficiency
c. Glucose Phosphate Isomerase Deficienc
d. Triosephosphate Isomerase, Hexokinase and
Diphosphoglycerate Mutase Deficiency

Answer 8

A. Pyruvate kinase deficiency

Question 9

PERIPHERAL SMEARS

Echinocytes

A. Pyruvate kinase deficiency
b. Pyrimidine-5-Nucleotidase (PN) Deficiency
c. Glucose Phosphate Isomerase Deficienc
d. Triosephosphate Isomerase, Hexokinase and
Diphosphoglycerate Mutase Deficiency

Answer 9

A. Pyruvate kinase deficiency

Question 10

✓ Fluorescent test- positive

A. Pyruvate kinase deficiency
b. Pyrimidine-5-Nucleotidase (PN) Deficiency
c. Glucose Phosphate Isomerase Deficienc
d. Triosephosphate Isomerase, Hexokinase and
Diphosphoglycerate Mutase Deficiency

Answer 10

A. Pyruvate kinase deficienc

Question 11

- Inherited
**- Caused by an abnormality in nucleotide ** metabolism

-** Acquired occurs in lead poisoning and
responsible for the basophilic stippling**

A. Pyruvate kinase deficiency
b. Pyrimidine-5-Nucleotidase (PN) Deficiency
c. Glucose Phosphate Isomerase Deficienc
d. Triosephosphate Isomerase, Hexokinase and
Diphosphoglycerate Mutase Deficiency

Answer 11

b. Pyrimidine-5-Nucleotidase (PN) Deficiency

Question 12

**This causes an abnormality in aerobic glycolysis causes a
moderately severe anemia
**

A. Pyruvate kinase deficiency
b. Pyrimidine-5-Nucleotidase (PN) Deficiency
c. Glucose Phosphate Isomerase Deficience
d. Triosephosphate Isomerase, Hexokinase and
Diphosphoglycerate Mutase Deficiency

Answer 12

c. Glucose Phosphate Isomerase Deficience

Question 13

Are other enzyme deficiencies that involve anaerobic
glycolysis

A. Triosephosphate Isomerase, Hexokinase and
Diphosphoglycerate Mutase Deficiency
B. Glutathione synthase, glutathione peroxidase and
glutathione reductase deficiencies

Answer 13

1. Triosephosphate Isomerase,
2. Hexokinase
3. Diphosphoglycerate Mutase Deficiency

A

Question 14

These enzymes are required in the HMP like G-6-PD,
hemolysis increase due to oxidant drug exposure

A. Triosephosphate Isomerase, Hexokinase and
Diphosphoglycerate Mutase Deficiency
B. Glutathione synthase, glutathione peroxidase and
glutathione reductase deficiencies

Answer 14

B. Glutathione synthase, glutathione peroxidase and
glutathione reductase deficiencies