RBC Disorders

Question 1

Erythrocytes in the fetus are larger than in adults, and at 22-23 wk gestation the mean corpuscular volume can be as high as ….

Answer 1

135 femtoliters (fL)

Question 2

How much is the normal ratio of HbA to HbA2 throughout life?

Answer 2

30 : 1

Question 3

Which of the following conditions is associated with increased Levels of HbA2>3.4%?

Answer 3

Megaloblastic anemia

Question 4

Microcytes are seen in blood films of which of the following conditions

Answer 4

A. Aplastic anemia B. Liver disease C. Anemia of chronic disease D. Down syndrome E. Hypothyroidism

Question 5

Basophil stippling is seen in blood films of which of the following conditions?

Answer 5

Thalassemia

Question 6

A 4-month-old infant presents with progressive pallor,microcephaly, snub nose, high-arched palate, hypertelorism, low-set ears, and triphalangeal thumb. Peripheral blood smear shows macrocytic RBCs with low reticulocyte count. Of the following, the MOST likely diagnosis is

Answer 6

Diamond-Blackfan anemia

Question 7

Which of the following is the mainstay therapy of Diamond-Blackfan anemia?

Answer 7

Corticosteroids

Question 8

Which of the following differentiate transient erythroblastopenia of childhood from iron-deficiency anemia?

Answer 8

MCV

Question 9

Which of the following is the BEST diagnostic test used to distinguish anemia of chronic disease (ACD) from iron-deficiency anemia (IDA)?

Answer 9

Soluble transferring receptor

Question 10

A 7-year-old child presents with pallor, jaundice, frontal bossing hepatosplenomegaly, , supernumerary toes, and gall stones revealed by US. Laboratory findings revealed hemoglobin 8 g/dL, MCV 110 fL, normoblasts, anisopoikilocytosis and basophilic stippling of RBCs. The bone marrow aspirate shows erythroid hyperplasia, megaloblastosis, with incompletely divided cells with thin chromatin bridges between nuclei of pairs of erythrocytes. Electron microscopy revealed erythroblasts with a characteristic “Swiss cheese” heterochromatin pattern. Of the following, the MOST likely diagnosis is

Answer 10

congenital dyserythropoietic anemia

Question 11

Which of the following provides very useful and early indicators of iron deficiency?

Answer 11

Reticulocyte hemoglobin concentration

Question 12

Iron therapy in developing countries may increase the virulence of certain

Answer 12

gram negative bacteria

Question 13

Which of the following RBC indices is MOST helpful in the diagnosis of hereditary spherocytosis?

Answer 13

MCHC

Question 14

Which of the following closely mimics hereditary spherocytosis in the neonatal period?

Answer 14

ABO incompatibility

Question 15

When splenectomy is indicated for hereditary spherocytosis, it should be performed after age of

Answer 15

6

Question 16

What is the MOST common inherited pancytopenia syndrome?

Answer 16

Fanconi anemia

Question 17

What is the MOST common congenital anomaly in Fanconi anemia?

Answer 17

Skeletal

Question 18

What is the latest hematological abnormality evolved in Fanconi anemia?

Answer 18

Anemia

Question 19

What is the MOST common available test for diagnosis of Fanconi anemia?

Answer 19

Chromosomal breakage test

Question 20

Which of the following malignancies is considered as a complication of Fanconi anemia?

Answer 20

AML

Question 21

What is the MOST common hematological abnormality in ShwachmanDiamond syndrome?

Answer 21

Neutropenia

Question 22

What is the MOST common non-hematological abnormality in ShwachmanDiamond syndrome?

Answer 22

Pancreatic insufficiency

Question 23

What is the usual physical finding in polycythemia?

Answer 23

Hepatosplenomegaly

Question 24

What is the MOST convenient initial treatment of polycythemia?

Answer 24

A. Phlebotomy

Question 25

What is the key reason that the nadir hemoglobin values of premature infants are lower than those of term infants?

Answer 25

Diminished plasma erythropoietin in response to anemia