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Chapter-06 Pathobiology, Prognostication and Management of Low-risk Myelodysplastic Syndrome

BOOK TITLE: Recent Advances in Hematology-3

Author
1. Pati HP
2. Rathi Shyam
ISBN
9788184488883
DOI
10.5005/jp/books/11240_6
Edition
1/e
Publishing Year
2011
Pages
10
Author Affiliations
1. All India Institute of Medical Sciences, New Delhi (India), All India Institute of Medical Sciences (AIIMS), Ansari Nagar, New Delhi, All India Institute of Medical Sciences (AIIMS), New Delhi, India, AIIMS, New Delhi, India, All India Institute of Medical Sciences, New Delhi, All India Institute of Medical Sciences, New Delhi, India
2. All India Institute of Medical Sciences, New Delhi, India
Chapter keywords

Abstract

The myelodysplastic syndrome (MDS) is considered to be a clonal disorder of stem cells, characterized by cytopenia (anemia, neutropenia and thrombocytopenia) with ineffective hematopoiesis and tendency for progression to leukemia. Defects within myeloid progenitors and bone marrow micrienvironment, both contribute to abnormal apoptosis, accumulation of cells with mutated DNA and progression to acute myeloid leukemia. International prognostic scoring system (IPSS) and world health organization prognostic scoring system (WPSS) are important tool for assessing the prognosis. Advances in understanding of pathobiology of MDS leads to development of newer treatment options. Hypocellular MDS especially those with young age, PNH clone and HLA DR 15 positive should be treated with immunosuppressive therapy. Patients with 5q-syndrome should be treated with lenalidomide. Hypomethylating agents or allogenic transplant should be reserved for patients refractory to primary therapy.

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