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Flashcards in MPN/MDS Deck (10)
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1
Q

What categories are present in the WHO Classification 2008?

A
  • RCC: Refractory cytopenia of childhood
  • RAEB: Refractory anemia with excess blasts
  • RAEB-T: Refractory anemia with excess blasts in transformation
  • MDS/MPN
2
Q

Define refractory cytopenia of childhood

A

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

3
Q

Define RAEB

A

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

4
Q

Define RAEB-T

A

PM and/or BM blasts 20-29%

5
Q

What differs between adult and childhood refractory cytopenia of childhood?

A

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

6
Q

What differs between adult and childhood RAEB?

A

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

7
Q

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

A
  1. Male sex
  2. Younger ager
  3. Plt count > 33*10^9
  4. Low age-adjusted Hgb F
8
Q

Inherited genetic predisposition for MDS

A
  • RUNX1, CEPBA (MDS/AML predisposition)

- GATA2

9
Q

What features are associated with GATA2 mutations?

A

“mono-mac syndrome” (absent macrophages and monocytes)

  • Immune deficiency
  • Infections: mycobacteria, HPV
  • Pulmonary alveolar proteinosis
  • Lymphedema (Emberger synd)
10
Q

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

A

Vasculitis