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期刊名称: Blood Reviews
Volume:21    Issue:5        Page:245-253

Hemophagocytic syndromes期刊论文

作者: Janka Gritta E

页码: 245-253
被引频次: 293
出版者: Elsevier Ltd,CHURCHILL LIVINGSTONE,Elsevier B.V
期刊名称: Blood Reviews
ISSN: 0268-960X
卷期: Volume:21    Issue:5
语言: English
摘要: Summary Hemophagocytic syndromes (hemophagocytic lymphohistiocytosis, HLH) represent a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. Biochemical markers include elevated ferritin and triglycerides, and low fibrinogen. Whereas in children several inherited immune deficiencies may lead to this syndrome, most adults with HLH have no known underlying immune defect. Nevertheless, impaired function of natural killer (NK) cells and cytotoxic T -cells (CTL) is characteristic for both genetic and acquired forms of HLH. Frequent triggers are infectious agents, mostly viruses of the herpes group. Malignant lymphomas, especially in adults, may be associated with HLH. A special form of HLH in rheumatic diseases is called macrophage-activation syndrome. Initially HLH may masquerade as a normal infection since all symptoms, even though less pronounced, may also be found in immune competent patients. Patients with HLH, however, cannot control the hyperinflammatory response which, if untreated, is fatal in genetic cases and in a high percentage of acquired cases. Awareness of the clinical symptoms and of the diagnostic criteria of HLH is important to start life-saving therapy with immunosuppressive/ immunomodulatory agents in time.
相关主题: Hematology, Oncology and Palliative Medicine, Hemophagocytosis, Review, Histiocytosis, Immune deficiency, Macrophage activation syndrome, immune deficiency, EBV INFECTION, LYMPHOHISTIOCYTOSIS, MALIGNANT HISTIOCYTOSIS, macrophage activation syndrome, histiocytosis, hemophagocytosis, GENE, review, JUVENILE RHEUMATOID-ARTHRITIS, IDIOPATHIC ARTHRITIS, STEM-CELL TRANSPLANTATION, HEMATOLOGY, EXPRESSION, T-CELLS, Splenomegaly, Diagnosis, Differential, Prognosis, Lymphohistiocytosis, Hemophagocytic - physiopathology, Inflammation Mediators - blood, Hematopoietic Stem Cell Transplantation, Lymphohistiocytosis, Hemophagocytic - diagnosis, Lymphohistiocytosis, Hemophagocytic - therapy, Fever, Cyclosporine - therapeutic use, Dexamethasone - therapeutic use, Hepatomegaly, Immunotherapy, Inflammation Mediators - metabolism, Lymphohistiocytosis, Hemophagocytic - genetics, Lymphomas,






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