Question 1

Which type of CDA is most common in Europe?

Accepted Answer

CDA II

Answer

Transcription-factor-related CDAs

Answer

CDA I

Answer

No particular type

Answer

CDA III

Question 2

What genetic mutations are primarily responsible for CDA I?

Accepted Answer

Homozygous mutations in CDAN1 or C15orf41

Answer

Mutations in KLF1

Answer

Mutations in KIF23

Answer

CDAN4

Answer

Mutations in SEC23B

Question 3

CDA II is characterized by which laboratory feature?

Accepted Answer

Binucleated late erythroblasts

Answer

Megaloblastoid maturation

Answer

Peripheral blood erythroblastosis

Answer

Giant multinucleated erythroblasts

Answer

Normal erythroblasts with abnormal iron homeostasis

Question 4

Which key finding is associated with CDA III patients?

Accepted Answer

Giant multinucleated erythroblasts

Answer

Hemolytic anemia symptoms

Answer

Macrocytic anemia with erythroblastemia

Answer

HEMPAS

Answer

Small bi-nucleated erythroblasts

Question 5

What is a common complication of congenital dyserythropoietic anemias?

Accepted Answer

Iron overload

Answer

Monoclonal gammopathy

Answer

Bone marrow failure

Answer

Secondary hemopoietic malignancy

Answer

Renal shutdown secondary to hemoglobinuria

Question 6

What is the inheritance pattern of CDA I?

Accepted Answer

Autosomal recessive

Answer

X-linked recessive

Answer

Mitochondrial inheritance

Answer

Autosomal dominant

Question 7

What is a distinguishing morphological feature of CDA I?

Accepted Answer

Internuclear chromatin bridges

Answer

Unconditional polychromasia

Answer

Single large nuclei

Answer

Absence of reticulocytosis

Answer

binuclearity

Question 8

The "Swiss cheese" appearance on electron microscopy is characteristic of:

Accepted Answer

CDA I

Answer

CDA IV

Answer

CDA II

Answer

CDA III

Question 9

Iron overload is least commonly observed in

Accepted Answer

CDA III

Answer

CDA I

Answer

CDA II

Answer

CDA IV

Question 10

CDA III is inherited in which of the following patterns?

Accepted Answer

Autosomal dominant

Answer

X-linked recessive

Answer

Autosomal recessive

Answer

De novo sporadic

Answer

Mitochondrial

Question 11

Which CDA subtype shows the highest association with monoclonal gammopathy and multiple myeloma?

Accepted Answer

CDA III

Answer

CDA variant

Answer

CDA II

Answer

CDA I

Answer

CDA IV

Question 12

Which therapy is uniquely useful in CDA I but not CDA II or III?

Accepted Answer

Interferon-alpha

Answer

Deferoxamine

Answer

Hematopoietic stem cell transplant

Answer

Splenectomy

Answer

Iron chelation

Question 13

Which type of anemia pattern is typically observed in CDA I?

Accepted Answer

Macrocytic anemia with reticulocytopenia

Answer

Normochromic normocytic anemia

Answer

Normocytic anemia with elevated reticulocytes

Answer

Microcytic hypochromic anemia

Answer

Hemolytic anemia with high bilirubin

Question 14

A 3-year-old girl is diagnosed with CDA II. She has elevated ferritin and normal T2 MRI. What is the best next management?

Accepted Answer

Start oral iron chelation therapy

Answer

Alpha interferon

Answer

Splenectomy

Answer

Immediate stem cell transplant

Answer

Discontinue transfusions

Question 15

A child with CDA II is being evaluated for persistent transfusion dependence. What intervention could reduce transfusion needs?

Accepted Answer

Splenectomy

Answer

IFN-alpha

Answer

Folic acid supplementation

Answer

Iron chelation

Answer

Hematopoietic growth factors

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