Deck 22: Myelodysplastic Syndromes

Question 1

Which of the following is considered an MDS/MPD variant in the WHO classification?

A)aCML
B)RARS
C)CML
D)PV

Answer 1

aCML

Question 2

The 5q-syndrome frequently shows dysplasia in which cell lineage?

A)Erythroid
B)Myeloid
C)Megakaryocytic
D)None of the above

Answer 2

Megakaryocytic

Question 3

A bone marrow specimen is received in the cytogenetics lab.FISH analysis has been ordered.The technologist performs the assay and notices that 96% of the patient cells contain both 5q- and 12q- deletions.These cytogenetic findings are characteristic of:

A)CML
B)AML
C)MDS
D)ALL

Answer 3

MDS

Question 4

All of the following are considered morphologic evidence of dyserythropoiesis except:

A)Abnormal degranulation.
B)Dimorphic cell population.
C)Oval macrocytes.
D)Karyorrhexis.

Question 5

Many of the myelodysplastic syndromes share a common cytogenetic abnormality.What is it?

A)Philadelphia chromosome
B)5q- deletion
C)t(8, 14)
D)t(15;17)

Question 6

Which WHO classification for MDS is best supported when the peripheral blood shows a general cytopenia, 5-19% blasts with Auer rods, and less than 1 x109 /L monocytes?

A)Refractory anemia (RA)
B)Refractory anemia with excess blasts 2
C)5Q-syndrome
D)Refractory cytopenia with multilineage dysplasia

Question 7

Which of the following IPSS scores has the highest survival rate in MDS?

A)Grade 0
B)Grade 0.5
C)Grade 1
D)Grade 1.5

Question 8

The WHO classification system for each subgroup of myelodysplastic syndrome is based on which characteristics?

A)Peripheral blood and bone marrow morphology
B)Cytogenetic abnormalities
C)Number of cytopenias
D)All of the above

Question 9

According to the WHO classification, how many blasts must be present in the bone marrow to classify a patient with acute leukemia?

A)19%
B)20%
C)30%
D)1%

Question 10

Which of the six WHO MDS subgroups contains two or more cytopenias, rare or no blasts, less than 1 x 10 9 /L monocytes in the peripheral blood and less than 5% blasts and less than 15% ringed sideroblasts in the bone marrow?

A)Refractory anemia (RA)
B)Refractory anemia with ringed sideroblasts (RARS)
C)Refractory cytopenia with multilineage dysplasia (RCMD)
D)Refractory anemia with excess blasts 2 (RAEB 2)

Question 11

Which of the following MDS/MPDclassifications includes the immunophenotype CD33, CD13 + MPO +?

A)CMML-1
B)CMML-2
C)aCML
D)JMML

Question 12

A patient blood smear shows oval macrocytes, a dimorphic population, and basophilic stippling.There are also 6% blasts in the peripheral blood.What is the presumptive classification based on these findings?

A)Refractory anemia
B)Refractory anemia with ringed sideroblasts
C)Refractory anemia with excess blasts -1
D)Refractory anemia with excess blasts -2

Question 13

What must be seen in the bone marrow to confirm refractory anemia with ringed sideroblasts?

A)A minimum of 30% ringed sideroblasts
B)A minimum of 5% ringed sideroblasts
C)A minimum of 5% monoblasts
D)A minimum of 15% ringed sideroblasts

Question 14

All of the following are included in the SHOMDS/MPD family except:

A)RARS.
B)CMML-1.
C)aCML.
D)JMML.

Question 15

The chromosomal abnormalities present in most cases of MDS are located on which of the following chromosomes?

A)4, 6, 8
B)3, 7, 12
C)5, 7, 9
D)5, 7, 8

Question 16

Myelodysplastic syndromes are sometimes called "pre-leukemias" because:

A)They have a progressive induction into acute leukemia.
B)They frequently progress to aplastic anemia.
C)Treatment for this disorder leads to leukemia.
D)They always precede leukemia

Question 17

Which of the following represents the granularity pattern of an abnormal in MDS?

A)Agranular
B)Hypogranular
C)Clumped granules
D)All of the above

Question 18

A 60-year-old patient visits his general practitioner because of fatigue and unexplained bruising.Physical examination revealed a frail, thin, pale man with widespread bruising.A CBC is ordered, and comes back abnormal.Peripheral blood smear examination revealed abnormal granularity of platelets and leukocytes.Red cells appear dimorphic.Bone marrow analysis is performed, and there were 20% ringed sideroblasts with dysplastic changes in RBC and WBC precursors.No cytogenetic abnormalities were detected.Based on this information, what is a probable diagnosis?

A)Acute leukemia
B)Chronic lymphocytic leukemia
C)Myelodysplastic syndrome
D)Myeloproliferative syndrome

Question 19

An unknown cell possesses the following characteristics: blast-like characteristics including open chromatin pattern, prominent nucleoli, absence of granules and a high N:C ratio.Based on this information, what is the most probable identity of the cell?

A)Type I blast
B)Type II blast
C)Type III blast
D)Promyelocyte

Question 20

A technologist is reviewing a blood smear, and finds a rare blast.Bone marrow analysis also shows dysplasia in 30% of the erythroblasts and myeloid precursors and 4% blasts.What is a possible diagnosis based on these findings?

A)RA
B)RAEB -1
C)RARS
D)R/cnd

Question 21

Which of the following laboratory tests would not be helpful in identifying MDS/MPD-u?

A)Immunophenotyping
B)Bone marrow analysis
C)Cytogenetic analysis
D)Molecular-based testing

Question 22

What is the major criterion for the diagnosis of acute leukemia?

A)The cytoplasm of the blasts contains no granules.
B)The nuclear cytoplasmic ratio in blasts is lower than that of type I blasts.
C)The blast count is less than 19% in the bone marrow.
D)The blast count is more than 20% in the bone marrow.

Question 23

Which test or tests support the theory that the abnormal cells in MDS are clones derived from an abnormal pluripotent stem cell?

A)Cytochemical studies
B)Peripheral blood smear examination
C)Bone marrow examination
D)Cytogenetic, G6PD isoenzyme, and molecular studies

Question 24

Give at least five morphologic indicators of dyserythropoiesis.

Question 25

Explain the relationship between myelodysplastic syndromes and acute leukemia.

Question 26

Which type of blasts contains fewer than 20 primary granules in the cytoplasm, has a centralized nucleus, and can be found in both AML and MDS?

A)Type I and II
B)Type I
C)Type II
D)Type III

Question 27

A 58-year-old male visits his physician for extreme fatigue.After conducting a standard investigation, the physician strongly suspects that his patient is suffering from CML.Which one of the following lab results would confirm this picture?

A)Hypercellular bone marrow
B)Hypercellular bone marrow with dysmyelopoiesis
C)Positive molecular analysis for BCR-ABL
D)Normal WBC count

Question 28

Patients with a low percentage of cells that express CD11b, and this marker have a shorter survival compared to patients whose cells do not have these characteristics?

A)CD7
B)CD8
C)CD4
D)CD31

Question 29

Explain the pathophysiology of MDS.

Question 30

A patient presents with general anemia and rare or no blasts.The bone marrow smear reveals 3% blasts, no Auer rods and 12% ringed sideroblasts.Which MDS classification is the associated with these findings?

A)Refractory anemia with ringed sideroblasts
B)Refractory anemia
C)Myelodysplastic syndrome, unclassified
D)5q-syndrome

Question 31

Which of the following tests would help differentiate between MDS/MPD disorders and CML?

A)CBC
B)PB smear
C)Cytogenetic analysis
D)LAP stain

Question 32

Explain the IPSS score system for prognosis and treatment of MDS.

Question 33

Explain how the FAB and WHO classifications of the MDS differ.