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Flashcards in MPN/MDS Deck (10)
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1

What categories are present in the WHO Classification 2008?

- RCC: Refractory cytopenia of childhood
- RAEB: Refractory anemia with excess blasts
- RAEB-T: Refractory anemia with excess blasts in transformation
- MDS/MPN

2

Define refractory cytopenia of childhood

Peripheral blood blasts <2% and/or BM blasts <5%

3

Define RAEB

PB blasts 2-19% and/or BM blasts 5-19%

4

Define RAEB-T

PM and/or BM blasts 20-29%

5

What differs between adult and childhood refractory cytopenia of childhood?

Unlike adults, childhood RCC often presents with hypocellular BM
(Can be difficult to differentiate from other causes of BM failure)

6

What differs between adult and childhood RAEB?

In childhood RAEB, unlike adults, counts can stay stable for years

7

Name 4 favorable prognostic factors in childhood JMML (other than genetic)

1. Male sex
2. Younger ager
3. Plt count > 33*10^9
4. Low age-adjusted Hgb F

8

Inherited genetic predisposition for MDS

- RUNX1, CEPBA (MDS/AML predisposition)
- GATA2

9

What features are associated with GATA2 mutations?

"mono-mac syndrome" (absent macrophages and monocytes)
- Immune deficiency
- Infections: mycobacteria, HPV
- Pulmonary alveolar proteinosis
- Lymphedema (Emberger synd)

10

What other features can be seen in cbl-related JMML?

Vasculitis