噬血细胞综合征21例临床分析并文献复习

发布时间：2018-12-20 18:13

【摘要】：目的:回顾性总结分析噬血细胞综合征(Hemophagocytic Syndrome,HPS)患者的临床特征,结合国内外文献进行综述及讨论,旨在提高各级各专业临床医师对该病的认识和诊断水平。方法:选取21例近5年(2011.1-2015.12)山西医科大学第二医院诊治的HPS患者,总结其病历资料(病因、临床表现、实验室化验指标、主要治疗策略及转归),对可能影响该病预后的因素进行初步探讨。结果:患者总数21例,其中男性9例(42.86%),女性12例(57.14%),男女二者之比是3:4;总体年龄分布区间为[18-76]岁,中位年龄为43岁。21例HPS患者最常见的病因是感染(14例),其中以单纯EBV感染最多见(10例);淋巴瘤及病因不明者分别为2例及5例。临床表现主要为:发热及脾大(19例),肺部感染(16例),肝大(12例),淋巴结肿大及浆膜腔积液分别为11例及10例;此外少见的有黄染、瘀点、瘀斑、皮疹,少数合并中枢神经系统症状、心功能不全、肾功能不全及消化道出血。实验室检查常见为:二系或三系血细胞减少(21例),20例行铁蛋白(SF)检查中有19例升高,肝功能损害(18例),骨髓噬血现象(17例),18例行NK细胞活性检查中有13例NK细胞活性下降/缺失;高甘油三脂血症(10例),低纤维蛋白原血症及凝血功能异常各占14例,3例合并DIC;1例行sCD25检查明显升高。治疗方法,积极控制原发病,主要治疗是激素、依托泊苷(VP16)和/或环孢素(CSA)治疗;选用CHOP方案化疗的是淋巴瘤患者(2例);4例患者应用大剂量甲强龙(500mg-1g/日)冲击治疗,1例血栓性血小板减少性紫癜(TTP)联合使用血浆置换。转归方面,9例患者早期好转出院,12例因病情加重死亡;死亡患者中,1例合并消化道出血,合并肺部感染及凝血功能异常各10例,3例合并DIC,5例脏器功能衰竭。比较早期好转组与死亡组患者的基本情况,包括初诊时的年龄、性别、主要并发症及实验室指标,发现两组在初诊时差异有统计学意义的指标为血红蛋白、SF、白细胞(WBC)及血小板(Plt)计数(所有P0.05)。结论:感染及肿瘤为继发性HPS常见病因。临床特征主要为发热、肝脾/淋巴结肿大、二系或三系血细胞减少、SF升高、NK细胞活性下降或缺失、骨髓噬血现象、低纤维蛋白原(FIB)血症,合并肝功能损害、肺部感染、凝血功能异常者占多数。早期诊断并及时治疗对改善疾病预后大有裨益。早期患者的WBC计数和Plt计数减少、贫血及SF明显升高可能与预后不良有关。影响该病预后的独立因素则是Plt计数减少。预后较差者多合并肝功能损害、凝血功能异常、肺部感染及脏器功能衰竭。
[Abstract]:Objective: to review and analyze the clinical features of hemophagocytic syndrome (Hemophagocytic Syndrome,HPS) patients, and to improve the understanding and diagnosis of hemophagocytic syndrome (Hemophagocytic Syndrome,HPS). Methods: 21 cases of HPS patients treated in the second Hospital of Shanxi Medical University in recent 5 years (2011.2015.12) were selected and their medical records (etiology, clinical manifestation, laboratory test index, main treatment strategy and outcome) were summarized. The factors that may affect the prognosis of the disease were preliminarily discussed. Results: there were 21 patients, including 9 males (42.86%) and 12 females (57.14%). The ratio of male to female was 3: 4. The overall age distribution was [18-76] years, with a median age of 43 years. The most common etiology of 21 patients with HPS was infection (14 cases), in which EBV infection was the most common (10 cases), lymphoma and unknown etiology were 2 cases and 5 cases, respectively. The main clinical manifestations were fever and splenomegaly (19 cases), pulmonary infection (16 cases), hepatomegaly (12 cases), lymphadenopathy and serous cavity effusion in 11 cases and 10 cases respectively. In addition, there are rare yellow stain, stasis spot, ecchymosis, rash, a few complicated with central nervous system symptoms, cardiac insufficiency, renal insufficiency and gastrointestinal bleeding. Laboratory tests were performed in 21 cases of second or third line hemopenia, 19 cases of elevated ferritin (SF), 18 cases of liver dysfunction, and 17 cases of hemophagocytosis of bone marrow. Among 18 cases of NK cell activity test, 13 cases of NK cell activity decreased / deleted; Hypertriglyceridemia (n = 10), hypofibrinogenemia (n = 14) and coagulation dysfunction (n = 14) were found in each group. SCD25 was significantly increased in 3 patients with DIC;1. The main treatment was hormone etoposide (VP16) and / or cyclosporine (CSA). Four patients were treated with high dose of methylenolone (500mg-1g/ day) and one patient with thrombotic thrombocytopenic purpura (TTP) was treated with plasma exchange. In terms of prognosis, 9 patients were discharged from hospital early and 12 died because of aggravation of the disease, among them, 1 case was complicated with gastrointestinal hemorrhage, 10 cases were complicated with pulmonary infection and abnormal coagulation function, and 3 cases were complicated with DIC,5 organ failure. The basic conditions of the patients in the early improvement group and the death group were compared, including age, sex, main complications and laboratory indexes at the time of first visit. The results showed that the difference between the two groups at the first visit was hemoglobin, SF,. WBC (WBC) and platelet (Plt) count (all P0.05). Conclusion: infection and tumor are the common etiology of secondary HPS. The main clinical features were fever, hepatosplenomegaly, decrease of blood cells in second or third line, increase of SF, decrease or absence of NK cell activity, hemophagocytosis of bone marrow, hypofibrinogen (FIB), liver function damage and pulmonary infection. The majority of patients had abnormal coagulation function. Early diagnosis and timely treatment are helpful to improve the prognosis of the disease. The decrease of WBC count and Plt count in early patients and the increase of anemia and SF may be related to poor prognosis. The independent factor influencing the prognosis of the disease was the reduction of Plt count. Poor prognosis was associated with liver dysfunction, abnormal coagulation, pulmonary infection and organ failure.
【学位授予单位】：山西医科大学
【学位级别】：硕士
【学位授予年份】：2016
【分类号】：R55

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