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Epstein-Barr Virus Infection-associated Hemophagocytic Syndrome

Epstein-Barr病毒感染合併噬血症候群

摘要


噬血症候群(hemophagocytic syndrome)又叫做噬血淋巴組織球增生(hemophagocytic lymphohistiocytosis),主要的臨床症狀是發燒、脾腫大、血球減少、高三酸甘油酯血症和一些病理學的發現,包括:在組織間可見廣泛的組織球(巨噬細胞和單核球)增生,這些增生的巨噬細胞有明顯吞噬其他血球,如紅血球、白血球和血小板,造成血球數減少。在臨床上,噬血症候群區分為原發性和續發性。續發性的噬血症候群續發於其他疾病,它與感染症,特別是病毒感染有著密不可分的關係;其他像是膠原血管疾病或是惡性腫瘤都有續發性噬血症候群的報告。藉由本例我們報告一位一歲八個月的女孩因Epstein-Barr病毒感染相關的續發性噬血症候群,並和文獻報告比較。

並列摘要


Hemophagocytic syndrome (HPS), also called hemophagocytic lymphohistiocytosis, is an unusual syndrome characterized by fever, splenomegaly, cytopenia, hypertriglyceridemia, and the pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone marrow and other tissue. Secondary HPS occurs after strong immunologic activation, such as systemic infection, autoimmune disease, or underlying malignancy. Virus infection associated HPS is called virus-associated hemophagocytic syndrome. We present a case of Epstein-Barr virus associated HPS and compare to the literature.

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