Current perspective in agnogenic myeloid metaplasia

Leukemia & Lymphoma
Ayalew Tefferi, M N Silverstein

Abstract

Agnogenic myeloid metaplasia (AMM) carries the worst prognosis among the chronic myeloproliferative disorders. Substantial bone marrow fibrosis, extramedullary hematopoiesis, anemia and hepatosplenomegaly are the characteristic features of the disease. AMM is currently incurable and the available treatment agents are mostly palliative and do not prolong life. Two pathogenetic processes are responsible for the impaired hematopoiesis and the clinical manifestations. The primary disease process is a clonal hematopoietic stem cell disorder which results in chronic myeloproliferation and atypical megakaryocytic hyperplasia. The secondary process of bone marrow fibrosis is the result of non-clonal fibroblastic proliferation and hyperactivity induced by growth factors abnormally shed from clonal megakaryocytes. Therefore, experimental treatment strategies may be directed towards either one or both of these disease processes. This report summarizes the current management options and new therapeutic endeavours.

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Citations

Oct 16, 2002·Cancer Genetics and Cytogenetics·Joris AndrieuxJean Luc Laï
Jan 20, 2000·Baillière's Clinical Haematology·A GeorgiiA Kreft
Aug 5, 2000·The Journal of Immunology : Official Journal of the American Association of Immunologists·P RameshwarP Gascon

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