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Myelodysplastic Syndrome

Myelodysplastic Syndrome. “brief overview”. Intro. MDS comprises a group of malignant stem cell disorders characterized by ineffective blood cell production and variable risk of transformation to acute leukemia. MDS subtypes. Refractory Anemia 21% (RA)

natalie-lucas
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Myelodysplastic Syndrome

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  1. Myelodysplastic Syndrome “brief overview”

  2. Intro • MDS comprises a group of malignant stem cell disorders characterized by ineffective blood cell production and variable risk of transformation to acute leukemia.

  3. MDS subtypes • Refractory Anemia 21% (RA) • RARS (refractory anemia w/ ringed sideroblasts 17%) • RA w/ excess blasts 37% (RAEB) • RA w/ excess blasts in transformation 12% (RAEB-T) • CMML (chronic myelomonoctic leukemia) 13%

  4. Prevalence • ~ 10,000 cases annually in US • 4.1 MDS / 2.1 AML per 100,000 • Risk of Development increases w/ Age • Unusual <50yrs, unless tx induced • Median age 65 or greater, w/ male predominance

  5. Predisposing Elements • Heritable – Genetic Disorders including Down’s, Congential Neutropenias, Fanconi’s Anemia, Ataxia Telangiectasia, NF-1…. • Acquired – Mutagen Exposure (alkylators, Topio II, Radioactive), Hemapoietic cell xplants, environmental exposures, Aplastic Anemia, PNH, Polycythemia Vera

  6. Clinical Presentation • Non-specific and Varied • Most asymptomatic and Dx found on labs • Fatigue, dizziness, weakness, “don’t feel well” • Less commonly Infxn, bleeding, bruising • Fever and wgt loss uncommon • Infxn is principal cause of death in MDS (neutropenia and granulocyte dysfunction) • Also rarely AI manifestations can occur

  7. Physical Findings • 60% Pale • 26% Petechaie and/or Purpura • HSPM and lymphadenopathy are uncommon save for CMML • Cutaenous Manifestations uncommon…but 2 recognized syndromes can occur in MDS --> Sweet’s – acute febrile neutrophilic dermatosis --> Myeloid Sarcoma – “chloroma”

  8. Laboratory • Bone Marrow and Blood changes variable, divided into FAB MDS subtypes. • Chromosomal Abnormalities are associated as well. • Anemia almost always present w/ low retic response • Pancytopenia in up to 50% of cases • <5% have isolated neutropenia or thrombocytopenia w/o anemia

  9. Differential Dx • Megaloblastic Anemia • Aplastic Anemia • Myelofibrosis • Atypical CML • HIV • Medications (VPA, Cellcept, Ganciclovir) • Etoh

  10. Our Patient… • Immunoglobin Production variably affected…Hypogammaglobulinemia 13%, polyclonal Hypergammaglobulinemia 30%, Monoclonal Gammopathy reported in 12% of patients • Lymphopenia largely 2/2 to reduced CD4+ cells inversely related to # of transfusions received. • RARS: <5% BM blasts, >15% ringed sideroblasts, accounts for ~17% of MDS

  11. Ringed Sideroblasts

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