Cytokine production by bone marrow mononuclear cells in inherited bone marrow failure syndromes
نویسندگان
چکیده
منابع مشابه
Inherited bone marrow failure syndromes: molecular features.
Recent advances resulting from the identification of the genes responsible for four inherited marrow failure syndromes, Fanconi anemia, dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome, are reviewed. The interpretation of genetic testing should be guided by an understanding of the limitations of such testing for each disorder. The possibility of an inherited basis...
متن کاملThe inherited bone marrow failure syndromes.
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...
متن کاملBone marrow failure syndromes.
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 ...
متن کاملBone marrow cell cycle markers in inherited bone marrow failure syndromes.
Patients with inherited bone marrow failure syndromes (IBMFS) are at increased risk of acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS), possibly related to cell cycle dysregulation. In a cross-sectional analysis of bone marrow from 77 IBMFS, 71 sporadic conditions (AML, MDS, acquired aplastic anemia) and 22 normal controls we found overexpression of p53 in IBMFS, AML, and MDS; o...
متن کاملTelomere length in inherited bone marrow failure syndromes.
Telomeres are long DNA repeats and a protein complex at chromosome ends that are essential for genome integrity. Telomeres are very short in patients with dyskeratosis congenita due to germline mutations in telomere biology genes. We compared telomere length in patients with Fanconi anemia, Diamond-Blackfan anemia and Shwachman-Diamond syndrome with telomere length in dyskeratosis congenita. Te...
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ژورنال
عنوان ژورنال: British Journal of Haematology
سال: 2013
ISSN: 0007-1048
DOI: 10.1111/bjh.12475