Myelodysplastic Syndromes Flashcards

1
Q

Group of acquired clonal hematologic disorder that affect maturation of erythroid, myeloid, and or megakaryocytic lines. PROGRESSIVE PB CYTOPENIAS (MAY EVENTUALLY PROGRESS TO AML)

A

Myelodysplastic Syndromes

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2
Q

Mechanism of Myelodysplastic Syndromes

A

Hypomethylation of DNA followed by hypermethylation of DNA

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3
Q

MDS peripheral blood findings

A
  • cytopenias
  • anemia (normochromic normocytic or macrocytic anemia)
  • dysplasia
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4
Q

MDS bone marrow findings

A
  • Hypercellular
  • Dysplasia
  • ineffective hematopoiesis
  • M:E ratio decreased
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5
Q

Is flow cytometry and cytogenetics diagnostic for MDS?

A

NO! important for providing prognostic risks and therapy but not diagnostic

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6
Q

Criteria for calling a ringed sideroblasts in the erythrocyte line

A

at least 5 iron granules that circle at least 1/3 of the nucleus

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7
Q

What are the most common lab findings in MDS?

A

macrocytic or normocytic anemia (with low retics)

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8
Q

MDS may present with _____ _____ RBCs but still have normal iron stores

A

microcytic hypochromic

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9
Q

MDS may present with megaloblastoid cellular development but still have _______ vit B12and folate values

A

normal

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10
Q

Most common myelodysplastic Syndromes (3)

A
  1. Refractory Anemia with Excess Blasts (RAEB-1 and 2)
  2. Refractory Cytopenia with Multilineage Dysplasia
  3. Refractory Cytopenia with Unilineage Dysplasia
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11
Q

PB Blasts in RAEB-1

A
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12
Q

PB Blasts in RAEB-2

A

5-19%

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13
Q

BM Blasts in RAEB-1

A

5-9%

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14
Q

BM Blasts in RAEB-2

A

10-19%

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15
Q

Myelodysplastic Syndrome with Isolated Del(5q)

  • what deletion
  • predominantly in what sex?
  • what anemia is characteristic
A
  • deletion of a 5q presumed tumor suppressor gene
  • women
  • macrocytic
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16
Q

RARS - refractory anemia with ringed sideroblasts

A

more than 15% of BM erythroid precursors are ringed sideroblasts

17
Q

How do you distinguish Myelodysplastic/Myeloproliferative Neoplasms from CML?

A

BCR-ABL1 Negative

18
Q

one specific type of a Myelodysplastic/Myeloproliferative Neoplasms

A

Juvenile Myelomonocytic Leukemia

19
Q

How do you score the prognosis for a myelodysplastic syndrome?

A

The International Prognosic scoring system IPSS

20
Q

The International Prognosic scoring system IPSS

- good and bad scores

A

0 to 2 and the higher number is bad

21
Q

Treatment: Myelodysplastic Syndrome

A

lenalidomide and farnesyltransferase inhibitors

22
Q

Cure for MDS

- what score and age to receive “cure”

A

Stem Cell Transplant

  • score greater than 2
  • less than 70 years old