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 1 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 2 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 3 MDS: Clinical Symptoms of Cytopenia Anemia > Neutropenia +/- anomegaly (infrequent) 4 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 5 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 6 7 8 MDS: Differential Diagnosis B12/folate deficiency Heavy metals (Arsenic) Congenital dyserythropoietic anemia Parvovirus B19 GCSF therapy (increased blasts) 9 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 10
