Myelodysplasia Flashcards
What are the common features of MDS?
clonal disorders of stem cells
ineffective haematopoiesis
- cytopaenias of any or all cell lines
risk of transformation to AML
- most common cause of anaemia in the elderly
What features give a poor prognosis?
Internation Prognostic Scoring system (IPSS)
Cytogenetics (7q-)
% bone marrow myeloblasts
no. of cytopaenias
age also poor prognostic factor
four risk groups: low intermediate 1 * intermediate 2 (OS 1.2 years) * high (OS 0.4 years)
- higher risk of transformation to AML
What cytogenetic abnormalities confer poor prognosis in MDS?
Multiple chromosomal abnormalities e.g.
+ 8
5q- (good prognosis)
7q- (poor prognosis)
What treatments are available for MDS?
transfusions +/- iron chelation (Exjade) EPO Drugs • Azacytidine (Vidaza) * HSC transplant *
- consider in high risk patients
What is 5q- syndrome?
Macrocytic anemia +/- thrombocytosis
Low risk of leukaemia transformation
biopsy shows micro-megakaryocytes, Peiger-Huet cells
2/3 transfusion dependent
What is Azacytadine?
Drug that causes demyethylation of tumour suppressor genes (works by affecting epigenetics)
- improves survival in MDS
- improves QOL
- delays transformation to AML
SE: local skin reactions
use in intermediate 2 and high risk patients
Who should be considered for allogeneic stem cell transplant in MDS?
young patients
consider in high risk patients
What are the usual FBC abnormalities in MDS?
macrocytic anaemia (with normal B12/folate)
What abnormalities can be found in the blood film in MDS?
macrocytosis
dyserythropoiesis
megaloblastic (nuclear to cytoplasmic development abnormal)
Peiger-Huet anomaly: Hypo granular neutrophils
What is the treatment for 5q- syndrome?
Responds to Lenalidomide
- 60% transfusion independence
Good prognosis - median survival = 10 years
