Myelodysplastic Syndromes Between FAB and WHO Mona F. Melhem, MD Professor, Department of Pathology University of Pittsburgh School of Medicine Chief Hematology VA Medical Center of Pittsburgh Pittsburgh, Pennsylvania Myelodysplastic Syndromes (MDS) Clonal hematopoietic Stem Cell Disease Dysplasia Ineffective Hematopoiesis (1 or more lines) Myeloblasts < 20% of all marrow cells Synonyms: Dysmyelopoietic syndromes Preleukemic syndromes Oligoblastic leukemia MDS: Epidemiology Older adults (median age: 70 years) Primary vs. Secondary MDS (S/P chemotherapy) Incidence: 3/100,000 non-age corrected 20/100,000 over age 70 MDS: Clinical Symptoms of Cytopenia Anemia > Neutropenia +/- anomegaly (infrequent) MDS: Etiology Primary No known history of toxic exposure Possible etiologies: Virus, Benzene, cigarette (2 fold risk), Fanconi anemia. Therapy-related Chemotherapy (alkylating agents) Radiation Therapy MDS: Morphology % blasts in marrow and blood Type and degree of dysplasia +/- ringed sideroblasts ic abnormalities del (5q) 500 cell diff in marrow 200 cell diff in PB MDS: Differential Diagnosis B12/folate deficiency Heavy metals (Arsenic) Congenital dyserythropoietic anemia Parvovirus B19 GCSF therapy (increased blasts) Myelodysplastic Syndromes FAB Classification RA RARS RAEB RAEB-T CMML WHO classification Myelodysplastic Syndromes RA RARS RCMD & RCMD-RS RAEB-1 & RAEB-2 MDS Unclassified MDS del(5q) Myelodysplastic/Myeloproliferative Diseases CMML Atypical CML Juvenile CMML MDS/MPD, unclassified
