Moon Jae Hyun, Hong Sung Pyo, Park Pil Won, Ko Kwang Hyun, Hwang Seong Gyu, Rim Kyu Sung, An Hee Jung, Kang Myung Seo
Department of Internal Medicine, Bundang CHA Hospital, Pochon CHA University, Yatap-dong, Bundang-gu, Seongnam, Korea.
Korean J Gastroenterol. 2006 Sep;48(3):205-9.
Reactive hemophagocytic syndrome or hemophagocytic lymphohistiocytosis, is characterized by the proliferation of benign histiocytes showing phagocytosis of blood cells in hematopoietic organs including bone marrow, spleen, or lymph nodes, accompanied by fever, hepatosplenomegaly, hepatic dysfunction, pancytopenia, and hypertriglyceridemia. The pathogenesis of reactive hemophagocytic syndrome is unknown. It is often associated with infection, malignant neoplasm, autoimmune disease, drugs and various immunodeficiencies. The prognosis of this syndrome is poor and the causes of death are hemorrhage, infection, or multiorgan failure. We experienced a case of hemophagocytic syndrome with terminal ileal ulcers, not associated with other causes. Thus, we report this case with a review of literatures.
反应性噬血细胞综合征或噬血细胞性淋巴组织细胞增生症，其特征为良性组织细胞增殖，这些组织细胞在包括骨髓、脾脏或淋巴结在内的造血器官中表现出对血细胞的吞噬作用，同时伴有发热、肝脾肿大、肝功能障碍、全血细胞减少和高甘油三酯血症。反应性噬血细胞综合征的发病机制尚不清楚。它常与感染、恶性肿瘤、自身免疫性疾病、药物及各种免疫缺陷相关。该综合征的预后较差，死亡原因是出血、感染或多器官功能衰竭。我们遇到一例伴有回肠末端溃疡的噬血细胞综合征病例，且与其他病因无关。因此，我们报告此病例并对文献进行综述。
