Myelodysplasia Flashcards
Myelodysplasia - description/characteristics/prognosis
Description
- heterogeneous group of myeloid neoplasms, evidene of myeloid dysplasia, predisposition to AML
Characterised by:
- Bone marrow failure (cytopenias - anaemia, thrombocytopenia, neutropenia)
- <20% blasts on peripheral blood/bone marrow
- risk of transformation to acute myeloid leukemia
Classification:
Refractory anaemia
Refractory anaemia with ringed sideroblasts
Refractory anaemia with multilineage dysplasia
Refractory anaemia with excess blasts 1 & 2 (<20%)
MDS unclassified
5q-syndrome: females, deletion of 5q only, better prognosis, treat with lenolidomide.
Chronic myelomonocytic leukemia (peripheral blood monocytes persistently >1 x 10^9/L)
Prognostic systems:
IPSS: based on cytogenetics, cytopenias, blast % (low/intermed/high)
WPSS: based on type of MDS, chromosomal changes, transfusion requirements (very low risk, LR, intermed risk, high risk, v high risk)
(refer to image for median survival and risk of leukemic transformation in 5yrs, for different risk levels of MDS)
Pathway mutations of splicing machinery in myelodysplasia https://www.nature.com/articles/nature10496
Myelodysplasia - treatment
Supportive care
- transfusion + iron chelating therapy (>20units)
Epigenetic therapy
- azacitidine, decitadine, or lenolidamide
Chemotherapy
- high dose for myeloid leukemia
Allogeneic stem cell transplant
- poor prognosis, high risk IPSS/very high risk WPSS
- suitable recipient, available donor
