RBC Disorders Flashcards
Erythrocytes in the fetus are larger than in adults, and at 22-23 wk gestation the mean corpuscular volume can be as high as ….
135 femtoliters (fL)
How much is the normal ratio of HbA to HbA2 throughout life?
30 : 1
Which of the following conditions is associated with increased Levels of HbA2>3.4%?
Megaloblastic anemia
Microcytes are seen in blood films of which of the following conditions
A. Aplastic anemia B. Liver disease C. Anemia of chronic disease D. Down syndrome E. Hypothyroidism
Basophil stippling is seen in blood films of which of the following conditions?
Thalassemia
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
Diamond-Blackfan anemia
Which of the following is the mainstay therapy of Diamond-Blackfan anemia?
Corticosteroids
Which of the following differentiate transient erythroblastopenia of childhood from iron-deficiency anemia?
MCV
Which of the following is the BEST diagnostic test used to distinguish anemia of chronic disease (ACD) from iron-deficiency anemia (IDA)?
Soluble transferring receptor
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
congenital dyserythropoietic anemia
Which of the following provides very useful and early indicators of iron deficiency?
Reticulocyte hemoglobin concentration
Iron therapy in developing countries may increase the virulence of certain
gram negative bacteria
Which of the following RBC indices is MOST helpful in the diagnosis of hereditary spherocytosis?
MCHC
Which of the following closely mimics hereditary spherocytosis in the neonatal period?
ABO incompatibility
When splenectomy is indicated for hereditary spherocytosis, it should be performed after age of
6
What is the MOST common inherited pancytopenia syndrome?
Fanconi anemia
What is the MOST common congenital anomaly in Fanconi anemia?
Skeletal
What is the latest hematological abnormality evolved in Fanconi anemia?
Anemia
What is the MOST common available test for diagnosis of Fanconi anemia?
Chromosomal breakage test
Which of the following malignancies is considered as a complication of Fanconi anemia?
AML
What is the MOST common hematological abnormality in ShwachmanDiamond syndrome?
Neutropenia
What is the MOST common non-hematological abnormality in ShwachmanDiamond syndrome?
Pancreatic insufficiency
What is the usual physical finding in polycythemia?
Hepatosplenomegaly
What is the MOST convenient initial treatment of polycythemia?
A. Phlebotomy
What is the key reason that the nadir hemoglobin values of premature infants are lower than those of term infants?
Diminished plasma erythropoietin in response to anemia
