Ch19 Myelodysplastic Syndromes

Question 0

The median age of MDS is:

Answer 0

70

Question 1

What is a heterogeneous group of clonal hematological malignancies characterized by cytopenias, dysplasia blood cells, and a propensity to transform into acute leukemia

Answer 1

Myelodysplastic syndrome (MDS)

Question 2

What is the hallmark of MDS?

Answer 2

Monoclonal derivation of cells

Question 3

What is the cell of origin in MDS

Answer 3

A pluripotent stem cell which is already committed to myeloid differentiation

Question 4

Programmed cell death is known as what?

Answer 4

Apoptosis

Question 5

What are the 3 stages of apoptosis

Answer 5

Initiation, commitment, execution

Question 6

How can apoptosis be initiated?

Answer 6

By chemotherapy, radiotherapy, corticosteroid, and growth factors

Question 7

MDS patients have an _______ rate of apoptosis

Answer 7

Increased

Question 8

MDS has an imbalance between growth-stimulatory and growth inhibitory cytokines that causes:

Answer 8

An increase in apoptosis

Question 9

What is the most common genetic anomaly in MDS?

Answer 9

Loss of genetic material - chromosomal deletion

Question 10

Phenotypical changes of MDS are:

Answer 10

Number of blast cells increase
Leukocyte sis may replace leukopenia
Enlarged spleen and liver
Proliferation of abnormal cells

Question 11

What type of anemia is present in at least 90% of MDS cases?

Answer 11

Macrocytic or normocytric

Question 12

In MDS RBC may have:

Answer 12

Pyruvate kinase deficiency and increased levels of HbF

Question 13

The survival rate of patients with MDS is:

Answer 13

Less than 2 years

Question 14

What is the only potential cure of MDS?

Answer 14

Allogeneic stem cell transplantation

Question 15

What type of anemia is most common in MDS?

Answer 15

Macrocytic and normochromic

Question 16

Which morphological abnormalities are helpful in the diagnosis of MDS?

Answer 16

Pseudo-pelger-Huët and hypogranulation
Ringed sideroblasts
Micromegakaryocytes, monolobular, megakaryocyte

Question 17

What cell surface antigens is of prognostic significance in MDS?

Answer 17

CD34

Question 18

What MDS subgroup has the most favorable prognosis?

Answer 18

RARS

Question 19

Which type of treatment may offer a cure in patients with MDS

Answer 19

Allogeneic BM transplantation

Question 20

What agent may lead to secondary MDS?

Answer 20

Alkylating agents

Question 21

What is most likely to affect the prognosis in MDS?

Answer 21

Increased BM myeloblasts

Question 22

What chromosomal abnormality is most often found in female patients with RA and elevated platelet counts?

Answer 22

5q-

Question 23

What biologic or genetic anomalies are important in the pathogenesis of MDS?

Answer 23

Chromosomal deletion a

Question 24

Which MDS scoring system is most often used today?

Answer 24

IPSS