Deck 19: Myelodysplastic Syndromes

Question 1

The French-American-British (FAB) classification system separates the myelodysplastic syndromes into __________ different types.

A) One
B) Three
C) Five
D) Six
E) None of the above

Answer 1

Five

Question 2

What is the purpose of transfusing granulocytes to a patient with RA?

A) Prevent hemorrhages
B) Prophylaxis to infection
C) Increase blood volume
D) Decrease plasma viscosity
E) None of the above

Answer 2

Prophylaxis to infection

Question 3

The most promising course of therapy for MDS is __________.

A) Bone marrow transplant
B) Androgens
C) Blood transfusion
D) Myelostimulants
E) None of the above

Answer 3

Bone marrow transplant

Question 4

The following morphologies are characteristic of dyserythropoiesis in MDS:

A) Basophilic stippling
B) Normochromic, normocytic red cells
C) Multinuclearity
D) A and C
E) A, B, and C

Question 5

RAEB contains a maximum of __________ blasts in the bone marrow.

A) 30%
B) 20%
C) 10%
D) 5%
E) None of the above

Question 6

Which is the most current system used for scoring MDS?

A) FAB score
B) International Prognostic Scoring System (IPSS)
C) Sanz
D) Bournemouth
E) None of the above

Question 7

Which factor(s) differentiate(s) RARS (refractory anemia with ringed sideroblasts) from refractory anemia?

A) Erythroid dimorphism
B) > 15% sideroblasts
C) Normal WBC
D) All of the above
E) None of the above

Question 8

The fundamental characteristic of MDS is:

A) Peripheral blood cytopenia and bone marrow hypocellularity
B) Peripheral blood cytopenia and bone marrow hypercellularity
C) Peripheral blood cytosis and bone marrow hypocellularity
D) Peripheral blood cytosis and bone marrow hypercellularity
E) None of the above

Question 9

Chromosomal abnormalities are directly correlated with the presence of the __________ population in patients with MDS.

A) Promyelocyte
B) Blast
C) Megakaryocyte
D) Erythrocyte
E) None of the above

Question 10

Which of the following is the most significant prognostic indicator in MDS?

A) Degree of cytopenia
B) Degree of dysplasia in peripheral blood
C) Percentage of bone marrow blasts
D) Degree of dysplasia in bone marrow
E) None of the above

Question 11

In calculating the percentage of myeloblasts in the bone marrow, the following data are given. Erythroid nuclear precursors (E) = 65%, myeloblasts = 14%, other cells = 21%. Based on these numbers, what is the percentage of myeloblasts by the FAB classification?

A) 14%
B) 30%
C) 40%
D) 60%
E) None of the above

Question 12

MDS occurs more often in which age group?

A) Pediatric
B) Child
C) Adult
D) Elderly
E) None of the above

Question 13

What specific features are characteristic of dysmegakaryocytopoiesis morphology in MDS (myelodysplastic syndrome)?

A) Macromegakaryocytes
B) Small platelets
C) Micromegakaryocytes
D) Normal segmentation
E) None of the above

Question 14

Which MDS classification has the best prognosis?

A) RAEB-T
B) RAEB
C) RA
D) CMML
E) None of the above

Question 15

Which type of MDS demonstrates a mild decreased white blood cell (WBC) count (3.9 $\times$ 10/L), increased erythropoiesis and normal numbers of blast cells in the marrow, and <15% sideroblasts?

A) Refractory anemia with ringed sideroblasts (RARS)
B) Refractory anemia (RA)
C) Refractory anemia with excess blasts (RAEB)
D) Chronic myelomonocytic leukemia (CMML)
E) None of the above

Question 16

Which specific features are characteristic of dysgranulopoiesis morphology in MDS?

A) Hypergranulation
B) Pure Pelger-Huët anomaly
C) Auer rods
D) Hypersegmentation in bone marrow
E) None of the above

Question 17

The myeloid-to-erythroid ratio in MDS:

A) Is increased
B) Is decreased
C) Represents normal hematopoiesis
D) Represents decreased erythropoiesis
E) None of the above

Question 18

The most common and consistent nonrandom chromosomal aberration in the MDS, refractory anemia is deletion of the long arm of __________.

A) Chromosome 16
B) Chromosome 5
C) Chromosome 6
D) Chromosome 9
E) None of the above

Question 19

Which MDS classification has the worst prognosis?

A) Refractory anemia with excess blasts in transformation (RAEB-T)
B) Refractory anemia (RA)
C) Chronic myelomonocytic leukemia (CMML)
D) Refractory anemia with excess blasts (RAEB)
E) None of the above

Question 20

Which of the following represents the "cornerstone" of myelodysplastic-syndrome treatment?

A) Component therapy
B) Antibiotic therapy
C) Autologous bone marrow transplant
D) Interferon
E) A and B

Question 21

Ringed sideroblasts (type III) are seen in what MDS classification?

A) RA
B) RAEB
C) RAEB-T
D) RARS
E) None of the above

Question 22

Which of the following agents may lead to secondary myelodysplastic syndrome (sMDS)?

A) Hydrocortisone
B) Alkylating agents
C) Irradiated blood components
D) Folic acid
E) Pyridoxal phosphate

Question 23

A type III sideroblast in dyserythropoiesis is demonstrated by:

A) 1-5 granules
B) Blue ring around the nucleus with Prussian blue
C) 5-10 granules
D) No granules
E) None of the above

Question 24

Which of the following biological and/or genetic anomalies are important in the pathogenesis of MDS?

A) Chromosomal translocations
B) Chromosomal deletions
C) Hemolysis
D) Increased intramedullary apoptosis
E) B and D

Question 25

Which of the following is most likely to affect prognosis in MDS?

A) Leukopenia
B) Increased bone marrow myeloblasts
C) Erythroid hyperplasia
D) Thrombocytosis
E) None of the above

Question 26

In female patients with RA and elevated platelet counts, which chromosome abnormality is most often found?

A) (5q-)
B) t(8;21)
C) (7q-)
D) t(15;17)
E) t(9;22)

Question 27

What characterizes the cytogenetic analysis of patients with MDS?

A) Translocations
B) Inversions
C) Deletions
D) Reciprocal translocations
E) All of the above

Question 28

Which of the MDS subtypes is more closely related to the chronic myeloproliferative disorders?

A) RAEB
B) CMML with WBC > 13 $\times$ 10⁹/L
C) CMML with WBC < 13 $\times$ 10⁹/L
D) RARS
E) None of the above