Question 1

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

Accepted Answer

Atypical chronic myeloid leukemia, BCR/ALB1- is considered an MDS/MPN variant by the World Health Organization's classification. Polycythemia vera is classified as a myeloproliferative neoplasm (MPN). Refractory anemia with ringed sideroblasts is classified as a myelodysplastic syndrome (MDS). Chronic myelogenous leukemia is classified as a myeloproliferative neoplasm (MPN) but is not considered a variant.

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?

Accepted Answer

The symptoms and laboratory findings suggest a myelodysplastic syndrome (MDS), which is characterized by dysplastic changes in blood cell precursors in the bone marrow. The abnormal granularity of platelets and leukocytes, as well as the dimorphic red cells, also suggest MDS. The presence of ringed sideroblasts in the bone marrow further supports this diagnosis. Acute leukemia and chronic lymphocytic leukemia would present with different laboratory findings and clinical features. Myeloproliferative syndrome would show an increase in the number of blood cells, which is not consistent with the patient's presentation.

Question 3

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

Accepted Answer

C

Question 4

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

Accepted Answer

Dysplastic cells in Myelodysplastic Syndromes (MDS) can exhibit various abnormalities in granulation, including agranular (lacking granules), hypogranular (reduced number of granules), and clumped granular patterns. This variability reflects the diverse nature of dysplasia in MDS.

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:

Accepted Answer

The presence of both 5q- and 12q- deletions in 96% of the patient's cells is characteristic of myelodysplastic syndrome (MDS). This cytogenetic abnormality is known as the "5q- syndrome" and is often seen in patients with MDS. CML (chronic myeloid leukemia) is characterized by the presence of the Philadelphia chromosome (a translocation between chromosomes 9 and 22), while AML (acute myeloid leukemia) and ALL (acute lymphoblastic leukemia) can have a variety of cytogenetic abnormalities, but not specifically the 5q- and 12q- deletions seen in this case.

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?

Accepted Answer

The subgroup that fits this description is Refractory cytopenia with multilineage dysplasia (RCMD) according to the World Health Organization's classification system of myelodysplastic syndromes (MDS).

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?

Accepted Answer

Oval macrocytes and basophilic stippling are characteristic of myelodysplastic syndromes (MDS), and the presence of a dimorphic population suggests a dyserythropoietic process. The 6% blasts indicate a progression of the MDS, placing the patient in the category of refractory anemia with excess blasts-1 (RAEB-1). The other options are not supported by the given findings.

Question 8

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

Accepted Answer

The most common chromosomal abnormalities in MDS involve deletions or other alterations of genetic material on chromosomes 5, 7, and 8.

Question 9

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

Accepted Answer

Myelodysplastic syndromes are characterized by one or more peripheral blood cytopenias, which may be anemia, neutropenia, or thrombocytopenia. The other choices are not defining characteristics of MDS.

Question 10

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

Accepted Answer

The answer of MDS associated with isolated del(5q)frequently shows dysplasia...

Question 11

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

Accepted Answer

The WHO classification system for each subgroup of myelodysplastic syndrome is based on (A) peripheral blood and bone marrow morphology, (B) cytogenetic abnormalities, and (C) number of cytopenias. Therefore, the correct answer is D, all of the above.

Question 12

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

Accepted Answer

IPSS Score 0 has the lowest risk category with a median survival of 5.7 years.

Question 13

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

Accepted Answer

The diagnosis of refractory anemia with ringed sideroblasts requires the presence of at least 15% ringed sideroblasts in the bone marrow. Therefore, option D is the correct choice. A minimum of 30% ringed sideroblasts is seen in refractory anemia with excess blasts, while the presence of monoblasts is indicative of acute myeloid leukemia, but not specifically refractory anemia with 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?

Accepted Answer

According to the WHO classification, a patient with acute leukemia must have at least 20% blasts present in their bone marrow to be classified as such. Therefore, the correct answer is option B. Options A and C are incorrect as they do not match the classification criteria. Option D is incorrect as it does not provide a valid percentage for blast presence.

Question 15

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

Accepted Answer

The answer of All of the following are included in...

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?

Accepted Answer

The WHO classification for MDS is based on the percentage of blasts in the bone marrow, cytogenetics, and presence of dysplasia in one or more cell lineages. In this scenario, the peripheral blood showing cytopenia and 5-19% blasts with Auer rods suggest a diagnosis of MDS associated with excess blasts (RAEB-2), the correct answer is B. A peripheral blood count with less than 1 × 10⁹/L monocytes does not fulfill the criteria for RA or RCMD. MDS associated with isolated del(5q) is characterized by the presence of an isolated deletion on chromosome 5q in the bone marrow cells and is typically associated with macrocytic anemia and normal or increased platelet counts. Refractory cytopenia with multilineage dysplasia (RCMD) requires dysplasia in at least 10% of cells in two or more lineages.

Question 17

Myelodysplastic syndromes are sometimes called preleukemias because:

Accepted Answer

Myelodysplastic syndromes (MDS) are sometimes called preleukemias because they have a higher risk of progressing to acute myeloid leukemia (AML). In fact, about 30% of patients with MDS may eventually develop AML, which is why MDS is considered a precursor or preleukemia.

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?

Accepted Answer

The characteristics described are typical of blast cells, which are immature precursor cells. The absence of granules and high N:C ratio suggests an agranular blast cell. The presence of prominent nucleoli is also a characteristic of blasts. Therefore, option A (agranular blast) is the most probable identity of the unknown cell. Option B (granular blast) is unlikely since the description mentions "absence" of granules. Option C (dysplastic promyelocyte) and option D (promyelocyte) are not consistent with the description, which mentions an open chromatin pattern, which is not typical of promyelocytes.

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?

Accepted Answer

The findings suggest refractory cytopenia with multilineage dysplasia (RCMD) due to the presence of dysplasia in more than 10% of cells in two or more myeloid lineages and less than 5% blasts in the bone marrow.

Question 20

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

Accepted Answer

Abnormal degranulation is actually a morphologic evidence of dysgranulopoiesis or abnormal development of granulocytes, not of dyserythropoiesis which pertains to abnormal development of erythrocytes. The other choices, dimorphic cell population, oval macrocytes, and karyorrhexis, are all morphologic evidence of dyserythropoiesis.

Quiz 25: Myelodysplastic Syndromes