期刊
BRITISH JOURNAL OF HAEMATOLOGY
卷 148, 期 2, 页码 205-216出版社
WILEY
DOI: 10.1111/j.1365-2141.2009.07991.x
关键词
cytopenia; autoimmune haemolytic anaemia; autoimmune neutropenia; lymphoproliferative disease; immune thrombocytopenic purpura
类别
资金
- United States Immunodeficiency Network [N01-A1-30070]
- Goldman Philanthropic Partnerships
- Rockefeller Brothers Fund
- Larry and Helen Hoag Foundation Clinical Translational Research Career Development
- Sanford Chair and Weinberg Fund of the Children's Hospital of Philadelphia
- NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES [N01AI030070] Funding Source: NIH RePORTER
P>Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of T cell dysregulation caused by defective Fas-mediated apoptosis. Patients with ALPS can develop a myriad of clinical manifestations including lymphadenopathy, hepatosplenomegaly, autoimmunity and increased rates of malignancy. ALPS may be more common that originally thought, and testing for ALPS should be considered in patients with unexplained lymphadenopathy, hepatosplenomegaly, and/or autoimmunity. As the pathophysiology of ALPS is better characterized, a number of targeted therapies are in preclinical development and clinical trials with promising early results. This review describes the clinical and laboratory manifestations found in ALPS patients, as well as the molecular basis for the disease and new advances in treatment.
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