Flashcards in MPN/MDS Deck (10)
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
Define refractory cytopenia of childhood
Peripheral blood blasts <2% and/or BM blasts <5%
PB blasts 2-19% and/or BM blasts 5-19%
PM and/or BM blasts 20-29%
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)
What differs between adult and childhood RAEB?
In childhood RAEB, unlike adults, counts can stay stable for years
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
Inherited genetic predisposition for MDS
- RUNX1, CEPBA (MDS/AML predisposition)
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)