Which of the following is a feature of type II congenital ...

[jc611]

Which of the following is a feature of type II congenital dyserythropoietic anemia type II (CDA II)?
 
  a. Giant, multinucleated red cell precursors
  b. Microcytic red cells
  c. Positive sucrose hemolysis test
  d. Negative acidified serum test (Ham test)

Question 2

Which of the following is an inherited disorder with pancytopenia, malformed kidneys, and mental deficiency?
 
  a. Myelodysplasia
  b. Fanconi anemia
  c. Diamond-Blackfan anemia
  d. Secondary aplastic anemia

[krakiolit]

Answer to Question 1

ANS: A
CDA II is also known as hereditary erythroblastic multinuclearity with positive acidified serum (HEMPAS). It is the most common type of CDA, and is, as the alternate name suggests, associated with giant, multinucleated erythroblastic precursors. The acidified serum test (Ham test) is positive, not the sucrose hemolysis test. Red cells are usually normocytic, although often anisocytosis is noted.

Answer to Question 2

ANS: B
Fanconi anemia is an autosomal recessive disorder, which means that the gene must be inherited from both parents to express the phenotype. Myelodysplasia and secondary aplastic anemia are both acquired. Diamond-Blackfan anemia is an inherited form of pure red cell aplasia (PRCA). Neutrophil and platelet precursors are normal.

[jc611]

Thank you

[krakiolit]

Happy to help you