Bone marrow failure syndromes.
نویسنده
چکیده
grelide in young patients with essential thrombocythemia. Blood 2001;97:863-6. 12 Harrison CN, Campbell PJ, Buck G, Wheatley K et al. A randomised comparison of hydroxyurea and anagrelide in highrisk essential thrombocythemia: the Medical Research Countil PT-1 Trial. N Engl J Med (in press). 13 Tefferi A. Myelofibrosis with myeloid metaplasia. Review. N Engl J Med 2000;342: 1255–65. 14 Tefferi A, Mesa RA, Nagorney DM, Schroeder G, Silverstein MN. Splenectomy in myelofibrosis with myeloid metaplasia; a single-institution experience with 223 patients. Blood 2000;95:2226–33.
منابع مشابه
Bone Marrow Failure Syndromes : An Overview
Bone marrow failure may be simply defined as pancytopenia (anemia, leukopenia, and thrombocytopenia, sometimes in various combinations) resulting from deficient hematopoiesis, (as against the cytopenias arising from peripheral destruction). This concept of bone marrow failure is imprecise but convenient.1,2 The classification shown in Table 1 is all-inclusive, and attempts to group the syndrome...
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Bone marrow failure syndromes (BMFS) are a cluster of inherited or acquired disorders characterized by peripheral cytopenia due to a decrease in hematopoietic progenitors or dysregulated hematopoiesis. Inherited bone marrow failure syndromes are mainly found in pediatric group, encompassing Diamond Blackfan anemia (DBA), Fanconi anemia (FA), congenital sideroblastic anemia (CSA), congenital neu...
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Molecular pathogenesis may be elucidated for inherited bone marrow failure syndromes (IBMFS). The study and presentation of the details of their molecular biology and biochemistry is warranted for appropriate diagnosis and management of afflicted patients and to identify the physiology of the normal hematopoiesis and mechanisms of carcinogenesis. Several themes have emerged within each subsecti...
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The treatment and medical management of aplastic anemia fundamentally differ between patients with inherited versus acquired marrow failure; however, the diagnosis of an inherited bone marrow failure syndrome is frequently obscure. Recent exciting advances in our understanding of the molecular pathophysiology of the inherited bone marrow failure syndromes have resulted in a profusion of new tes...
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BACKGROUND Inherited bone marrow failure syndromes are rare genetic disorders characterized by bone marrow failure, congenital anomalies, and cancer predisposition. Available single disease registries provide reliable information regarding natural history, efficacy and side effects of treatments, and contribute to the discovery of the causative genes. However, these registries could not shed li...
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Congenital bone marrow failure syndromes (BMFSs) are relatively rare disorders characterized by aberrant development in one or more hematopoietic lineages. Genetic alterations have now been identified in most of these disorders although the exact role of the molecular defects has yet to be elucidated. Most of these diseases are successfully managed with supportive care, however, treatment refra...
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عنوان ژورنال:
- Clinical medicine
دوره 5 4 شماره
صفحات -
تاریخ انتشار 2005