22) MDS Flashcards

1
Q

primary characteristics of MDS

A
  • PB cytopenia
  • cellular maturation abnormalities (dyspoiesis)
  • BM hyperplasia
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2
Q

excessive apoptosis in the BM

A

MDS

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3
Q

MDS incidence

A

most common >50 years
60% males
3-13 per 100,000

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4
Q

most common finding in MDS

A

anemia

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5
Q

the only cell that might be ↑ in MDS is…

A

monos

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6
Q

MDS usually leads to a ———– anemia

A

macrocytic (similar to megaloblastic)

may be dimorphic pop

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7
Q

will NOT be present in granulocytes with MDS

A
  • toxic gran
  • dohle bodies
  • vacuolization
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8
Q

signs of dysgranulopoiesis

A
  • agranular/hypogranular
  • bluer cytoplasm
  • pseudo-PH
  • hypersegmentation
  • donut shape nucleus
  • bright pink granules
  • dense blue rim in cytoplasm
  • Auer rods
  • ↓ enzyme activity
  • functional defects
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9
Q

signs of dysthrombopoiesis

A
  • giant plts
  • hypogranular plts
  • large granules
  • micromegakaryocytes
  • abnormal megakaryocyte nucleus shape
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10
Q

in MDS, …… in BM ↑ risk of leukemia

A

central clusters of myeloid precursors

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11
Q

———- hyperplasia most common in MDS

A

erythroid

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12
Q

signs of dyserythropoiesis

A
  • megaloblastoid nucleus/asynchrony
  • giant, multinucleated erythroblasts
  • nuclear budding
  • karryhorhexis
  • vacuoles or stippling
  • ringed sideroblasts
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13
Q

iron, B12, folate in MDS

A
  • serum iron normal or ↑
  • TIBC normal or ↓
  • B12/folate normal or ↑
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14
Q

dysgranulopoiesis may lead to blasts with…

A

few granules

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15
Q

MDS-SLD

A

MDS with single lineage dysplasia

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16
Q

cytopenia in 1+ cell lines, dysplasia in only 1 cell line

A

MDS-SLD

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17
Q

most common MDS-SLS

and others

A

refractory anemia

refractory neutropenia
refractory thrombocytopenia

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18
Q

refractory anemia from MDS-SLD is usually…

A

normocytic or macrocytic

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19
Q

MDS-RS

A

MDS with ringed sideroblasts

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20
Q

2 types of MDS-RS

A
  • MDS-RS-SLD
  • MDS-RS-MLD
21
Q

SF3B1

A

MDS-RS

22
Q

requirements for MDS-RS

A

5-15% RS and SF3B1 mutation
OR
>15% RS

23
Q

MDS-MLD

A

MDS with multilineage dysplasia

24
Q

dysplasia in at least 10% of cells in 2+ lineages

A

MDS-MLD

25
Q

30-40% of all MDS cases

A

MDS-MLD

26
Q

MDS-MLD may progress to…

A

AML

27
Q

MDS-EB

A

MDS with excess blasts

28
Q

blast count >5% in BM, cytopenia in 2+ lineages, dysplasia in all 3 lineages

A

MDS-EB

29
Q

most aggressive MDS and likely to progress to acute leukemia

A

MDS-EB

30
Q

2 types of MDS-EB

A
  • MDS-EB1: 5-9% blasts in BM, no auer rods
  • MDS-EB2: 10-19% blasts in BM, auer rods
31
Q

MDS with good prognosis and low risk of progression

70% cases are women

A

MDS with isolated del(5q)

32
Q

deletion of long arm of chromosme 5

A

MDS with isolated del(5q)

33
Q

sign of MDS with isolated del(5q) in PB/BM

A

hypolobulated megakaryocytes

34
Q

3 types of MDS-U

A
  • with 1-4% PB blasts
  • with single lineage dysplasia
  • based on defining cytogenetic abnormality
35
Q

MDS of childhood involving persistent cytopenias and dysplasia in any or all lines

A

refractory cytopenia of childhood

36
Q

proliferation + dysplasia

A

MDS/MPN

37
Q

monocytosis, far left shift, and absence of BCR/ABL1

A

CMML

38
Q

↑ lysozyme (from monos)

A

CMML

39
Q

> 20% monos in BM

A

CMML

40
Q

types of CMML

A
  • CMML1: <10% blasts in BM
  • CMML2: 10-19% blasts in BM
41
Q

CML with no detectable BCR/ABL1 and dysplasia

A

aCML

42
Q

aCML

A

atypical CML

43
Q

CMML in children
no BCR/ABL1

A

JMML

44
Q

JMML

A

juvenile myelomonocytic leukemia

45
Q

75% dx in children <3yo
can be in kids 1 month to adolescent

A

JMML

46
Q

MDS/MPN-RS-T

A

MDS/MPN with ringed sideroblasts and thrombocytosis

47
Q

↑↑ plt
>15% ringed sideroblasts
JAK2 mutation

A

MDS/MPN-RS-T

48
Q

only cure for MDS

A

BM transplant