Hemophagoctic lymphohistiocytosis--recent concept.

摘要: Hemophagocytic lymphohistiocytosis is a rare condition characterized by highly stimulated but inactive immune response. The disease may be inherited or acquired due to infections, collagen vascular diseases and malignancies. pathological hallmark of the syndrome aggressive proliferation macrophages histiocytes. Decreased NK cell activity results in increased T activation resulting production large quantities interferon gamma (IFN gamma), tumor necrosis factor alpha (TNF alpha) granulocyte macrophage colony stimulating (GM-CSF). This causes sustained tissue infiltration as well interleukin 1 (IL1) 6 (IL6).The inflammatory reaction extensive damage associated symptoms. Patients with HLH commonly present high fever, anemia splenomegaly. Minimal diagnostic parameters are complete hemogram, liver function test, serum triglycerides ferritin, coagulation profile including fibrinogen bone marrow aspiration. Two sensitive marker an plasma concentration chain soluble IL2 receptor (CD25) impaired activity. Hyperinflammation can treated steroid, Cyclosporine prevents lymphocytes immunoglobulin infusion helps control infection. Etoposide life saving specially case Ebstein Barr Viruses Histiocyte Society 1994 developed common treatment protocol (HLH-94). In January 2004 revised was opened entitled HLH-2004, which based on HLH-94 minor modifications. There remission rate HLH-2004 protocols.