Deck 25: Myelodysplastic Syndromes

Question 1

Which of the following does the World Health Organization's classification consider an MDS/MPN variant?

A)Polycythemia vera
B)Refractory anemia with ringed sideroblasts
C)Chronic myelogenous leukemia
D)Atypical chronic myeloid leukemia,BCR/ALB1-

Answer 1

Atypical chronic myeloid leukemia,BCR/ALB1-

Question 2

A 60-year-old patient visited his general practitioner because of fatigue and unexplained bruising.Physical examination revealed a frail,thin,pale man with widespread bruising.A CBC was ordered and revealed abnormal granularity of platelets and leukocytes.Red cells appeared dimorphic.Bone marrow analysis showed 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

Answer 2

Myelodysplastic syndrome

Question 3

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

A)Chronic myelomonocytic leukemia-1
B)Chronic myelomonocytic leukemia-2
C)Atypical chronic myeloid leukemia,BCR/ABL1-
D)Juvenile myelomonocytic leukemia

Answer 3

Atypical chronic myeloid leukemia,BCR/ABL1-

Question 4

Which of the following represents the granule pattern of a dysplastic cell in MDS?

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

Question 5

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

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

Question 6

Which of the World Health Organization's MDS subgroups contains two or more cytopenias,rare or no blasts,<1 × 10⁹/L monocytes in the peripheral blood,<5% blasts,and <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 7

A patient blood smear shows oval macrocytes,a dimorphic population,and basophilic stippling.The peripheral blood also has 6% blasts .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 8

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 9

Which of the following is a defining characteristic of myelodysplastic syndromes (MDS)?

A)One or more peripheral blood cytopenias
B)The presence of BCR/ABL1
C)Overproliferation of all myeloid elements
D)Dyslymphopoiesis

Question 10

MDS associated with isolated del(5q)frequently shows dysplasia in which cell lineage?

A)Erythroid
B)Myeloid
C)Megakaryocytic
D)Monocytes

Question 11

On which characteristic is the WHO classification system for each subgroup of myelodysplastic syndrome based?

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

Question 12

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

A)0
B)0.5
C)1.0
D)1.5

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

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

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

Question 15

All of the following are included in the MDS/MPN family except:

A)Refractory anemia with ringed sideroblasts
B)Chronic myelomonocytic leukemia
C)Atypical chronic myeloid leukemia,BCR/ALB1-
D)Juvenile myelomonocytic leukemia

Question 16

Which World Health Organization (WHO)classification for MDS is best supported when the peripheral blood shows a general cytopenia,5-19% blasts with Auer rods,and <1 × 10⁹ /L monocytes?

A)Refractory anemia
B)Refractory anemia with excess blasts 2
C)MDS associated with isolated del(5q)
D)Refractory cytopenia with multilineage dysplasia

Question 17

Myelodysplastic syndromes are sometimes called preleukemias 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 18

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

A)Agranular blast
B)Granular blast
C)Dysplastic promyelocyte
D)Promyelocyte

Question 19

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

A)RA
B)RAEB-1
C)RARS
D)RCMD

Question 20

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

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

Question 21

Which of the following laboratory tests would not be helpful in identifying MDS/MPN,U?

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

Question 22

Which of the following is a likely diagnosis in a 55-year-old male with a bone marrow cellularity of 22%?

A)Therapy-related myelodysplasia
B)MDS with fibrosis
C)Myelodysplastic syndrome,unclassifiable
D)Hypoplastic MDS

Question 23

Explain the relationship between myelodysplastic syndromes and acute leukemia.

Question 24

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 and molecular studies

Question 25

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 <19% in the bone marrow.
D)The blast count is >20% in the bone marrow.

Question 26

Give at least five morphologic indicators of dyserythropoiesis.

Question 27

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,unclassifiable
D)MDS associated with isolated del(5q)

Question 28

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

A)Agranular blast
B)Granular blast
C)Dysplastic promyelocyte
D)Promyelocyte

Question 29

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

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

Question 30

Explain the International Prognostic Scoring System (IPSS)score system for prognosis and treatment of MDS.

Question 31

Explain the pathophysiology of MDS.

Question 32

Which of the following characteristics are associated with a poor prognosis for MDS?

A)25% bone marrow blasts
B)Normal karyotypes
C)No cytopenias
D)<5% marrow blasts

Question 33

A 61-year-old woman saw her primary care physician because of fatigue and dyspnea on exertion for the past 3 months.Past medical history noted that the patient had been treated 5 years earlier with the DNA alkylating agent cyclophosphamide,non-Hodgkin lymphoma.A CBC revealed the following:
WBC: 3.6* 10⁹/L
RBC: 2.8 * 10₁₂ /L
Hb: 9.1 g/dL
MCV: 104 fL
Reticulocyte count: 2.0%
Normal vitamin B₁₂ and folic acid levels
PLT: 120 * 10⁹/L
Differential: 6% blasts
A bone marrow biopsy revealed hypercellularity,and dyshematopoiesis of all three myeloid lineages.
Suggest an appropriate follow-up test and a likely diagnosis for this patient.

Question 34

A 58-year-old male visits his physician for extreme fatigue.After conducting a standard investigation,the physician strongly suspects that the patient is suffering from CML.Which 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/ABL1
D)Increased WBC count