Usera: Myelodysplastic Syndromes

Question 1

A GROUP OF CLONAL HEMATOPOIETIC STEM CELL DISEASES CHARACTERIZED BY ONE OR MORE PERIPHERAL BLOOD CYTOPENIA(S), WITH DYSPLASIA IN ONE OR MORE MYELOID LINES.

Answer 1

myelodysplastic syndromes

Question 2

Who gets myelodysplastic syndromes typically?

Answer 2

older adults (~70yo males)

Question 3

What is classification of myelodysplastic syndromes based on?

Answer 3

cytopenias (reduction in blood cell number)
degree of dysplasia in peripheral blood and bone marrow
blast count

Question 4

What is this:

Peripheral blood: anemia, no blasts
Bone marrow: dysplastic erythroids, <15% ringed sideroblasts

Answer 4

refractory anemia

Question 5

What is this:

Peripheral blood: anemia, dimorphic red cell population, pappenheimer bodies, no blasts
Bone marrow: dysplastic erythroids, 15% ringed sideroblasts

Answer 5

refractory anemia with ringed sideroblasts (RARS)

Question 6

What is this:

Peripheral blood: Bi-or pancytopenia, <5% blasts, no Auer rods, 5-19% blasts or Auer rods
Bone marrow: 5-9%blasts, no Auer rods, 10-19% blasts or Auer rods

Answer 6

Refractory anemia with excess blasts (RAEB 1 or RAEB 2)

Question 7

What is this:

Peripheral blood: anemia, normal to increased platelets
Bone marrow: hypolobated megakaryocytes, isolated 5q karyotype

Answer 7

MDS with del(5q)

Question 8

What causes myelodysplastic syndromes?

Answer 8

abnormal stem cell clone proliferates; initating stimuli are unknown but involve genetic damage

Question 9

What are the clinical findings/symptoms of myelodysplastic syndromes?

Answer 9

fatigue/weakness
palor
ecchymoses (bruising)
hemorrhage
infection

Question 10

What are some laboratory findings in the peripheral blood for erythrocytes in myelodysplastic syndrome?

Answer 10

anemia
macrocytosis
oval macrocytes
anisocytosis (different sizes)
poikilocytosis (different shapes)
nucleated RBCs
decreased reticulocytes

Question 11

What are some laboratory findings in the peripheral blood for granulocytes in myelodysplastic syndromes?

Answer 11

leukopenia
neutropenia
left-shift
abnormal granules
hypersegmentation
ring nuclei

Question 12

What are some laboratory findings in the peripheral blood for platelets in myelodysplastic syndromes?

Answer 12

thrombocytopenia
thrombocytosis
giant platelets
hypogranular platelets
abnormal granulation

Question 13

Myelodysplastic syndromes frequently involve which chromosomes?

Answer 13

5 and 7

Question 14

Does isolated 5q syndrome have a good prognosis? Do complex karyotypes (>3 abnormalities) have a good prognosis?

Answer 14

yes; no

Question 15

What is the median survival for pts with myelodysplastic syndrome? What are two factors that make medial survival worse?

Answer 15

<2 yrs; increasing blast percentage and trilineage dysplasia

Question 16

Options for treatment of myelodysplastic syndrome?

Answer 16

Supportive care
RBC transfusion for anemia
Platelets for bleeding prophylaxis
Antibiotics for infections, neutropenia
Growth factors (GM-CSF, EPO)
Chemotherapy for poorer prognosis
Bone marrow transplantation

Question 17

What has been shown to decrease blood transfusion requirements and retard the progression of MDS to AML?

Answer 17

chemotherapy w/ hypomethylating agents

5-azacytidine and decitabine