haematology - myelodysplastic syndromes

Question 1

define myelodysplastic syndromes

Answer 1

heterogeneous group of progressive disorders

ineffective proliferation and differentiation of abnormally maturing myeloid SCs

<20% blasts

Question 2

myelodysplastic syndromes typically sen in

Answer 2

elderly

Question 3

myelodysplastic syndromes characteristics

Answer 3

peripheral cytopenia
abnormal cell maturation
risk of AML transformation

Question 4

clinical features of myelodsyplastic syndromes

Answer 4

infection, bleeding, fatigue

hyper cellular BM

Question 5

blood film in myelodysplastic syndromes

Answer 5

RBCs - ring sideroblasts

WBCs - hypo granulation, Pseudo-Pelger-huet anomaly

Platelets - micromegakaryocytes, hypolobated nuclei

Question 6

treatment of myelodysplastic syndromes

Answer 6

supportive - transfusions, EPO, G-CSF, abx

biological modifiers - immunosuppressive drugs, lenalidomide, azacytidine

chemotherapy - similar AML

allogeneic SCT

Question 7

prognosis of myelodysplastic syndromes

Answer 7

depends on:
international prognostic scoring system
BM blast %
karyotype
degree of cytopenia

mortality rule of 3: 1/3 from infection, 1/3 bleeding, 1/3 acute leukaemia