Zhang Ya-Li, Hao Jing-Nan, Sun Meng-Meng, Xing Xiao-Ying, Qiao Shu-Kai
General Medical Department, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China.
Department of Hematology, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Aug;32(4):1230-1237. doi: 10.19746/j.cnki.issn.1009-2137.2024.04.040.
To understand the etiology, clinical characteristics and prognosis of secondary hemophagocytic syndrome (HLH), so as to improve the understanding of HLH and reduce the rates of misdiagnosis and missed diagnosis of HLH.
A retrospective study was conducted to analyze the cause, clinical characteristics, laboratory findings, therapy and outcomes of 75 adult patients with secondary HLH admitted to our hospital from January 2015 to December 2021. Follow-up continued until the last discharge time.
Among 75 patients, infection-related HLH was the most common (45.33%), followed by lymphoma-related HLH (17.33%). Fever was the most common clinical manifestation (97.67%). Laboratory indicators such as NK cell activity (98.31% low or absent), sCD25 (93.22% increased), and serum ferritin (94.44% elevated) had higher sensitivity in diagnosis. By comparing the clinical manifestations and laboratory indicators of HLH patients with different causes, sex, lymph node enlargement and bone marrow morphology were more valuable for the diagnosis of primary disease (all <0.05). By comparing the treatment and clinical outcomes of HLH patients with different causes, the highest clinical remission rate (83.3%) was achieved in patients with autoimmune disease-related HLH treated with hormone+cyclosporine ( <0.05). The overall 12-month survival rate of all patients was 26.7%, in which the infection-related HLH was the lowest (14.7%) while autoimmune disease-related HLH was the highest (63.6%).
The causes and clinical characteristics of adult secondary HLH are varied, with poor prognosis and heterogeneity in disease severity. It is important to identify HLH cause early for diagnosis and needed to further understand HLH.
了解继发性噬血细胞综合征（HLH）的病因、临床特点及预后，以提高对HLH的认识，降低HLH的误诊和漏诊率。
采用回顾性研究方法，分析2015年1月至2021年12月我院收治的75例成人继发性HLH患者的病因、临床特点、实验室检查结果、治疗方法及预后。随访至最后一次出院时间。
75例患者中，感染相关HLH最为常见（45.33%），其次为淋巴瘤相关HLH（17.33%）。发热是最常见的临床表现（97.67%）。NK细胞活性（98.31%降低或缺失）、sCD25（93.22%升高）、血清铁蛋白（94.44%升高）等实验室指标在诊断中具有较高的敏感性。比较不同病因HLH患者的临床表现和实验室指标，性别、淋巴结肿大及骨髓形态对原发病诊断价值更大（均P<0.05）。比较不同病因HLH患者的治疗及临床结局，激素+环孢素治疗的自身免疫病相关HLH患者临床缓解率最高（83.3%，P<0.05）。所有患者12个月总生存率为26.7%，其中感染相关HLH最低（14.7%），自身免疫病相关HLH最高（63.6%）。
成人继发性HLH病因及临床特点多样，预后差，病情严重程度存在异质性。早期明确HLH病因对诊断至关重要，仍需进一步加深对HLH的认识。
