Internal Medicine

0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:17

1028 Myelodysplastic Syndrome

MYELODYSPLASTIC SYNDROME

D. GARY GILLILAND, MD, PhD

history & physical

History

■Majority of cases are sporadic

■Risks: exposure to chemical solvents (e.g., benzene) or pesticides; sig-

nificant exposure to ionizing radiation or chemotherapy (alkylating

agents, topoisomerase inhibitors). Risk increases with age (median

age at diagnosis approximately 65).

■Incidence approximately 1/100,000 per year all ages; 50/100,00 per

year over 65

■High risk of progression to acute myelogenous leukemia (AML)

Signs & Symptoms

■Related to cytopenia of one or more hematopoietic lineages

■Neutropenia: bacterial infections (presentation with opportunistic

infections rare)

■Anemia: fatigue, dyspnea on exertion, or angina in older patients

■Thrombocytopenia: easy bruisability, epistaxis, petechiae

tests

Basic Blood Tests

■CBC: neutropenia, anemia or thrombocytopenia. Absolute lympho-

cyte count usually normal

■Blood smear:

➣Granulocytes: poorly granulated, hyposegmented, Pelger-Huet

anomaly

➣Red cells: hypochromic, polychromasia, teardrops, nucleated

RBC

■Mild macrocytosis (MCV 100–105)

■Platelets: large, megakaryocyte fragments may be present

■Specific Diagnostic Tests:

■Bone marrow aspiration and biopsy:

➣BONE MARROW DYSPLASIA IS DIAGNOSTIC HALLMARK OF

MDS

■Dysmyelopoiesis: large primary granules, decreased granules num-

bers, bizarre nuclear forms, Pelger-Huet cells.