13例噬血细胞综合征的临床特征分析

目的：分析噬血细胞综合征（HPS）患者的临床特征，进一步提高对本病的认识。方法：回顾性分析13例HPS患者的临床资料。结果：13例患者均表现为持续性或间歇性高热，均有脾肿大，2例为巨脾，9例同时肝肿大；4例患者发病时伴随消化系统症状；13例患者均表现为血象3少或2少；7例有高甘油三酯血症，4例有低纤维蛋白原血症；8例患者出现铁蛋白升高，其中4例高于2000ug／L，并伴有多器官功能衰竭；4例患者进行外周血流式细胞术分型，NK细胞均明显低于正常；所有患者骨髓象中均发现组织细胞增多，比例为2．O％～18．0％，存在吞噬血小板和红细胞现象；13例检查了EBV相关抗体，其中12例IgG，1例IgM和IgG抗体均为阳性；有6例患者进行了化疗，其中5例使用VP方案，1例使用CHOP方案化疗6个疗程，病情缓解；2例患者未进行化疗，病情好转。结论：HPS常呈动态发展过程，在发病初期极易误诊，应积极进行骨髓细胞学、免疫分型及可溶性白细胞介素-2受体（sL-2R）水平等检查，努力寻找病因和诱因；部分诊断指标可能在疾病发展过程中才显现，早期治疗有助于改善预后。

Abstract：

Objective.. To analyze the clinical features of patients with hemophagocytic syndrome （HPS）and enhance understanding of the disease further. Methods.. Clinical data of 13 patients diagnosed with HPS were analyzed retrospectively. Results： Thirteen patients showed persistent or intermittent high fever, splenomegaly, two cases with massive splenomegaly, nine cases had hepatomegaly at the same time. Four cases had the symptom of gastrointestinal system; 13 cases showed less for the blood cells, as three or two less; 7 cases with hypertrigliceridemia, 4 cases with hypofibrinogenemia. Ferritin increased in 8 cases, and 4 above 2000ug/L, 4 cases developed to multiple organ failure; four cases were examined for the peripheral blood flow type, their NK cells were significantly lower than normall increased histiocytosis in bone marrow were found in all patients, ratio from 2. 0% to 18. 0%, phagocytosis of platelets and red blood cells in the histiocytes were common; 13 cases were examined EBV-related antibodies, 12 cases were IgG, 1 case was IgM and IgG antibodies positive; 6 cases were treated with the chemotherapy, of which 5 cases used the VP regimen, one case used CHOP with 6 courses, then got remission; patients had no chemotherapy, and improved too. Conclusions.. HPS often has the dynamic development process, easily misdiag nosed in early stages of disease. The examinations of bone marrow cytology, flow immunophenotyping and sL-2R level should be carried out actively, and try to search for the causes and incentives. Some diagnostic criteria may become apparent during the development of the disease. Early treatment can help improve patiats＇ prognosis.

Clinical Characteristics of 13 Cases With Hemophagocytic Syndrome

Objective.. To analyze the clinical features of patients with hemophagocytic syndrome （HPS）and enhance understanding of the disease further. Methods.. Clinical data of 13 patients diagnosed with HPS were analyzed retrospectively. Results： Thirteen patients showed persistent or intermittent high fever, splenomegaly, two cases with massive splenomegaly, nine cases had hepatomegaly at the same time. Four cases had the symptom of gastrointestinal system; 13 cases showed less for the blood cells, as three or two less; 7 cases with hypertrigliceridemia, 4 cases with hypofibrinogenemia. Ferritin increased in 8 cases, and 4 above 2000ug/L, 4 cases developed to multiple organ failure; four cases were examined for the peripheral blood flow type, their NK cells were significantly lower than normall increased histiocytosis in bone marrow were found in all patients, ratio from 2. 0% to 18. 0%, phagocytosis of platelets and red blood cells in the histiocytes were common; 13 cases were examined EBV-related antibodies, 12 cases were IgG, 1 case was IgM and IgG antibodies positive; 6 cases were treated with the chemotherapy, of which 5 cases used the VP regimen, one case used CHOP with 6 courses, then got remission; patients had no chemotherapy, and improved too. Conclusions.. HPS often has the dynamic development process, easily misdiag nosed in early stages of disease. The examinations of bone marrow cytology, flow immunophenotyping and sL-2R level should be carried out actively, and try to search for the causes and incentives. Some diagnostic criteria may become apparent during the development of the disease. Early treatment can help improve patiats＇ prognosis.