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Hematology > Myelodysplastic Syndromes > Flashcards

Myelodysplastic Syndromes Flashcards

1
Q

Myelodysplastic Syndromes (MDS) definition

A

stem cell disorders characterized by pancytopenia w/ maturation abnormalities

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2
Q

2 types of MDS

A

primary (de novo) - most of all idiopathic

secondary to therapy - chemo / radiation

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3
Q

Etiology of MDS

A

result from proliferation of abnormal cells - affects myeloid line mutation may be caused by chemical exposure, radiation or viral infection

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4
Q

Epidemiology of MDS

A

>50 yrs (median 70)

slight male predominance

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5
Q

Symptoms of MDS

A

asymptomatic

anemia symptoms

thrombocytopenia symptoms

neutropenia symptoms

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6
Q

Cellular Abnormalities of MDS

A

dysplasia including dyserythropoiesis, dysmyelopoiesis, dysmegakaryopoiesis

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7
Q

Dyserythropoiesis

A

defective development of RBCs

most common are oval macrocytes

MACRO, NORMO classification

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8
Q

Dysmyelopoiesis

A

abnormal granulation, hyposegmentation, possible left shift in the neutrophil line

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9
Q

Dysmegakaryopoiesis

A

giant platelets, abnormal platelet granulation small or abnormal megakaryocytes

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10
Q

Lab findings of MDS in PB

A

cytopenias & dysplasia (abnormal everything)

macro, normo anemia

oval macrocytes

RBC inclusions & NRBCs

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11
Q

Lab findings of MDS in BM

A

hypercellular!

all cell lines exhibit dysplasia

giant nucleated RBC precursors

ringed sideroblasts!

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12
Q

FAB classification of MDS

A
  1. Refractory Anemia (RA)
  2. Refractory anemia w/ ringed sideroblasts (RARS)
  3. Refractory anemia w/ excess blast (RAEB)
  4. Chronic myelomonocytic leukemia (CMML)
  5. Refractory anemia with excess blasts in transformation (RAEB-t)
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13
Q

Refactory Anemia (RA) - FAB

A

blasts in PB -<1%

blasts in BM- <5%

ringed sideroblasts - <15%

monos in BM - normal

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14
Q

RA with ringed sideroblasts (RARS) - FAB

A

blasts in PB- 1%

blasts in BM-<5%

ringed sideroblasts- 15%!!!

monos in BM - normal

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15
Q

RA with excess blasts (RAEB) - FAB

A

blasts in PB- <5%!!

blasts in BM - 5-20%!!!

ringed sideroblasts - variable

monos in BM - increased!!

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16
Q

RAEB in transformation (RAEB-t) - FAB

A

blasts in PB- >5%!!!!

blasts in BM - 21-30%!!!

ringed sideroblasts - variable

monos in BM - normal

17
Q

Chronic Myelomonocytic leukemia (CMML) - FAB

A

blasts in PB- >5%

blasts in BM-<20%!

ringed sideroblasts- variable

monos in BM - normal

18
Q

WHO classification of MDS

A
  1. RA
  2. RARS
  3. Refractory cytopenia w/ multilineage dysplasia (RCMD)
  4. RAEB (1 and 2)
  5. MDS associated w/ isolated del (5q syndrome)
  6. MDS, unclassified (MDS-U)
19
Q

RA - WHO

A

PB- anemia, rare or no blasts

BM- Erythroid dysplasia only, <15% ringed sideroblasts

20
Q

RARS - WHO

A

PB - anemia, rare or no blasts

BM - Erythroid dysplasia only, 15% ringed sideroblasts (!!)

21
Q

RCMD - WHO

A

PB - cytopenias, rare or no blasts

BM - multilineage dysplasia, <15% ringed sideroblasts

22
Q

RAEB 1 -WHO

A

PB - cytopenias, <5% blasts

BM - unilineage or multilineage dysplasia, 5-9% blasts!!

23
Q

RAEB-2 WHO

A

PB- cytopenias, 5-19% blasts, AUER RODS PRESENT

BM- unilineage or multilineage dysplasia, 10-15% blasts!!!!, AUER RODS PRESENT

24
Q

5q- syndrome WHO

A

PB - anemia, normal or INCREASED PLT, <5% blasts Isolated deletion of 5q cytogenetic abnormality

25
Q

MDS - U WHO

A

PB- cytopenias, rare or no blasts

BM- unilineage dysplasia, <5% blasts

26
Q

Variants of MDS

A
  1. hypoplastic MDS
  2. Therapy related MDS
  3. Childhood MDS
27
Q

Hypoplastic MDS

A

the 10% of MDS cases that have hypocellular BM

difficult to distinguish from aplastic anemia

28
Q

Therapy related MDS

A

MDS secondary to chemo/ radiation

highest incidence occurs 3-8 years after treatment

29
Q

Childhood MDS

A

few cases have been reported

probably more but not correctly diagnosed

30
Q

Cytogenetic Abnormalities that contribute to MDS

A

additional copy of chromosome 21

addition on chromosome 8

loss on chromosome 7

inversion on chromosome 3

del 5q

Hematology (14 decks)

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