MYELODYSPLASTIC SYNDROMES 2009. Marion Sternbach, MD, FRCP(C) FACP. Myelodysplastic Syndromes ( MDS ). Definition: Myelo = marrow in Greek Dys = irregular in Greek Plasia = proliferation in Greek
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Marion Sternbach, MD, FRCP(C)FACP
Dys = irregular in Greek
Plasia = proliferation in Greek
MDS is a heterogenous stem cell disease with a very active and abnormal proliferation of hematopoiesis in the bone marrow with asynchronous and delayed maturation of the different cell lines and early apoptosis ( cell death ) leading to ineffective hematopoiesis and peripheral blood (PB) cytopenia.
e.g. defic. Chemotaxis of granuloc., bleeding, etc.
erythroblast with still immature
Alk. PO4-ase granules
Nucleus with visible
Chromosomes ready to
Sideroblasts ( RAS ) Idiopathic Myelofibrosis with
Excess of Blasts ( RAEB )
Excess of Blasts in transformation (CML)
( RAEB – T )
Acute Myeloid or Monocytic leuk.
2005 – 51 year old fire fighter previously healthy,
Exposed to the large tire fire in Hagersville in late 90 – ies.
Ref. for fatigue and Pancytopenia.
Past Hx. Not contributory. Family Hx : no neoplasias.
Funct. Enq. : fatigue and easy bruising.
Phys. Exam: tall, fit, slim. No nodes, no hep-spl.megaly. Lung, CVS, abdomen, CNS – intact.
Lungs clear, CVS – Reg.S1-S2, systolic ejection M.2/6 over entire precordium.
MSK, CNS – intact. Integument – ecchymoses on shins.
Hb.-108, MCV- 92 Lc -3.2, N- 0.6, Ly – 1.2, Mono – 1.2, Blasts – 0.4, Plat. -76 X 10^9 / l .
B12 , folate – N., Creatinine – 75, Ferritin – 700,
Urine – RBC-s in sediment. LFT- N., ANA – Neg.,
Coombs’ – Neg.
B.M – Myeloid hyperplasia, blasts – 12%, Hemosiderin – ring sideroblasts.
Cytogenetics – multiple nonspecific anomalies.
Diagnosis – RAEB –T
Therapy: Allopurinol, Cytosar subcut., + 6TG.
Went in less than 3 mos. Into AMML , refractory, died.
Crrackles at both lung bases.
Uric Acid – 550 .
These type of MDS may respond to immunosuppression ( steroids and ATG )
AA treated may recover with clonal hematopoiesis, develop PNH, MDS and finally AML
Cytotoxic T - cell
Death receptors : Fas, TNF-R
Lenalidomide ( Revlimid ) – very effective in 5q- syndrome.
(RAEB) – 3 ; one 5q –
LAP = 183 ( Normal up to 130 )
Thank You apoptosis in B.M.
Thank you for your attention