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Myelodysplastic Syndrome

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Presentation on theme: "Myelodysplastic Syndrome"— Presentation transcript:

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 RA <5 <1 No RAS Yes RAEB 5-20 +- CMML <20 RAEB-T 21-30 >5
TYPE BM Blasts% Peripheral blood blasts % Auer Rods Monocytes > 1000 /uL Ringed Sideroblasts >15% RA <5 <1 No RAS Yes RAEB 5-20 +- CMML <20 RAEB-T 21-30 >5

5 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

6 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

7 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

8 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”

9 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

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

11 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

12 Ringed Sideroblasts

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