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目的:探讨成人噬血细胞综合征(HPS)的临床特点,旨在进一步提高对该病的认识。方法:回顾分析19例成人HPS的临床资料。结果:19例患者中男11例,女8例;中位发病年龄34(18~61)岁。考虑肿瘤相关5例(26.3%),感染相关5例(26.3%),结缔组织病4例(21.1%),原因不明5例(26.3%)。19例患者(100%)均有高热,14例(73.7%)表现为肝脾肿大,5例(26.3%)表现为多发浅表淋巴结肿大,3例(15.8%)出现皮疹,3例(15.8%)出现神经系统症状。19例患者均出现血清铁蛋白及血清乳酸脱氢酶升高,18例(94.7%)可见血细胞减少,14例(73.7%)血清甘油三酯水平升高,5例(26.3%)出现低纤维蛋白原血症,12例(63.2%)出现肝功能异常。4例(21.1%)EB病毒DNA滴度升高。全部患者于骨髓中发现吞噬细胞现象。全组患者8例存活,7例死亡,4例失访。19例患者的生存时间为0.3~39.8个月(中位生存时间为4.7个月),病死率36.8%。结论:HPS是一种少见疾病,其病因复杂,进展迅速,病死率高,且发病机制尚未明确,提高对HPS的认识,做出早期诊断和及时的治疗至关重要。
Objective: To investigate the clinical features of adult hemophagocytic syndrome (HPS) and to further improve the understanding of the disease. Methods: The clinical data of 19 adult HPS patients were retrospectively analyzed. Results: There were 11 males and 8 females in 19 cases. The median age of onset was 34 (18 ~ 61) years old. Five cases (26.3%) were considered related to cancer, 5 (26.3%) were related to infection, 4 (21.1%) had connective tissue disease and 5 (26.3%) were unknown. Nineteen patients (100%) had hyperthermia, 14 (73.7%) had hepatosplenomegaly, 5 (26.3%) had multiple superficial lymph nodes, 3 (15.8%) had rash and 3 (15.8%) had neurological symptoms. Serum ferritin and serum lactate dehydrogenase were elevated in all 19 patients. Cytopenia was observed in 18 (94.7%) patients, elevated serum triglycerides in 14 patients (73.7%), and low fibrosis in 5 patients (26.3%) Alzheimer’s disease, 12 cases (63.2%) had liver dysfunction. Four cases (21.1%) EBV DNA titer increased. All patients found in the bone marrow phagocytic phenomenon. All patients survived in 8 cases, 7 died and 4 lost. The survival time of 19 patients was 0.3-39.8 months (median survival time was 4.7 months), the mortality rate was 36.8%. Conclusions: HPS is a rare disease with complex etiology, rapid progression, high mortality rate and unclear pathogenesis. It is very important to improve HPS awareness, make early diagnosis and prompt treatment.