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目的分析噬血细胞综合征(HPS)患儿的临床特点,以提高对本病早期诊断,减少误诊。方法回顾性分析41例 HPS 患儿的病因、临床症状、体征、实验室检查结果及转归。结果本组病例以持续高热(100%)、肝脏肿大(97.6%)及脾脏肿大(95.1%)为突出表现,其余表现为淋巴结肿大(65.9%),呼吸系统症状(53.7%),多浆膜腔积液(26.8%),黄疸(26.8%),中枢神经系统症状(14.6%),消化道出血(12.2%),皮疹(12.2%)。实验室检查以肝功能损害最为突出(100%),以酶学改变(LDH、AST、ALT、GGT)及低白蛋白血症为主;其次为骨髓有噬血细胞现象(92.7%)、全血细胞减少(70.7%)、凝血功能障碍(52.4%)、DIC、高甘油三酯血症及低钠血症等。病因分析显示,感染相关性 HPS(IAHS)占63.4%,以 EB 病毒相关性噬血细胞综合征(EBV-AHS)最多(占 IAHS 的69.2%)。死亡14例中,IAHS 11例(EBV-AHS 9例),非 IAHS 3例(恶性淋巴瘤1例)。对 LDH 和 AST 水平与病死率作相关分析,发现 LDH 和 AST 升高水平与病死率呈正相关(r 值分别为0.486和0.516,P 值均<0.05)。年龄<3岁、LDH 水平>2000 U/L 和 AST 水平>200 U/L 是预后不良的危险因素(P 值分别为0.031、0.002和0.001)。结论 HPS 可由多种病因所致,临床表现多样;EBV-AHS 预后凶险,病死率高;年龄、LDH 和 AST 水平是 HPS 预后的影响因素;多次骨髓检查有助于及时诊断。
Objective To analyze the clinical features of children with hemophagocytic syndrome (HPS) to improve the early diagnosis of the disease and reduce the misdiagnosis. Methods Retrospective analysis of etiology, clinical symptoms and signs, laboratory findings and outcome of 41 HPS children. Results The patients were characterized by persistent high fever (100%), enlarged liver (97.6%) and splenomegaly (95.1%). The remaining manifestations were swollen lymph nodes (65.9%), respiratory symptoms (53.7% Multiple serous effusions (26.8%), jaundice (26.8%), central nervous system symptoms (14.6%), gastrointestinal bleeding (12.2%) and rash (12.2%). Laboratory tests showed the most prominent liver damage (100%), with enzyme changes (LDH, AST, ALT, GGT) and hypoalbuminemia, followed by bone marrow hemophagocytosis (92.7%), whole blood cells Reduce (70.7%), coagulation disorders (52.4%), DIC, hypertriglyceridemia and hyponatremia. Etiological analysis showed that infection-associated HPS (IAHS) accounted for 63.4% of the total, and Epstein-Barr virus-associated hemophagocytic syndrome (EBV-AHS) was the largest (69.2% of IAHS). In the 14 cases of death, 11 cases of IAHS (9 cases of EBV-AHS) and 3 cases of non-IAHS (1 case of malignant lymphoma). Correlation analysis between LDH and AST levels and mortality showed that LDH and AST levels were positively correlated with the case fatality rate (r = 0.486 and 0.516, P <0.05 respectively). Age <3 years, LDH level> 2000 U / L and AST level> 200 U / L were risk factors for poor prognosis (P values were 0.031, 0.002 and 0.001, respectively). Conclusions HPS may be caused by a variety of etiologies, with various clinical manifestations. The prognosis of EBV-AHS is dangerous and the mortality rate is high. Age, LDH and AST levels are the influencing factors of HPS prognosis. Multiple bone marrow biopsy can be helpful for timely diagnosis.