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Myelodysplastic Syndrome

Etiology, Prognosis and Management

  • Therapy in Practice
  • Published:
American Journal of Cancer

Abstract

The myelodysplastic syndromes (MDS) are a heterogeneous group of stem cell malignancies characterized by dysplastic and ineffective hematopoiesis and a variable risk of transformation to acute myeloid leukemia (AML). They usually occur in elderly people with an age at diagnosis of between 60 and 75 years. The natural history of these syndromes ranges from a chronic form spanning years [refractory anemia with or without ring sideroblasts (RARS)] to forms with a rapid evolution to AML [refractory anemia (RA) with excess blasts (RAEB), and refractory anemia with excess blasts in transformation (RAEB-t)]. Although the relationship between de novo AML and MDS has been controversial, recent evidence suggests that AML (especially AML in the elderly) and MDS are part of the same continuous disease spectrum rather than distinct disorders. MDS are clonal diseases with a step-wise genetic progression leading to secondary acquired, MDS specific-cytogenetic aberrations characterized by gains and loss of specific chromosomal regions. The treatment options for patients with MDS are complex and highly individualized, although, most therapies are poorly effective and the curative approaches are only available for aminority of patients.

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Acknowledgements

Dr Neumeister is supported by an Erwin Schrödinder Fellowship of the Austrian Fond Wissenschaftlicher Forschung (FWF).

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Authors and Affiliations

  1. Division of Hormone-Dependent Tumor Biology, Department of Molecular and Developmental Biology, The Albert Einstein Cancer Center, Albert Einstein College of Medicine, New York, New York, USA

    Peter Neumeister & Richard Pestell

  2. Division of Hematology of Karl Franzens University, Graz, Austria

    Peter Neumeister, Gerald Jaeger, Werner Linkesch & Heinz Sill

  3. Department of Internal Medicine of Karl Franzens University, Graz, Austria

    Beate Balent

  4. The Albert Einstein Cancer Center, Albert Einstein College of Medicine, Chanin 302A, D, 1300 Morris Park Ave, New York, NY, 10461, USA

    Peter Neumeister

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Neumeister, P., Pestell, R., Balent, B. et al. Myelodysplastic Syndrome. Am J Cancer 1, 301–311 (2002). https://doi.org/10.2165/00024669-200201050-00001

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