CAJ | 학술논문

目的：探讨噬血细胞综合征(HPS)的临床特点、诊断、治疗及临床预后因素。方法回顾性分析了26例噬血细胞综合征病因、临床特点、实验室检查、治疗与转归。结果发热、淋巴结肿大、肝脾肿大、皮肤瘀斑、黄染是最常见的临床表现。全血细胞减少(100.0%)、血清铁蛋白升高(61.5%)、血清乳酸脱氢酶增高(96.2%)、谷草转氨酶/谷丙转氨酶/总胆红素升高(73.1%)、凝血功能异常(65.4%)、甘油三酯增多(34.6%)是本组病例实验室特点；骨髓象：有核细胞增生大多活跃,均可见成熟组织细胞和噬血细胞。16例肿瘤相关性HPS,7例感染相关性HPS及3例原因不明HPS。结论 HPS可由多种原因引起,临床表现复杂多样,临床预后差,应积极寻找原发病因并对患者进行个体化治疗,有可能减少病死率。
목적：탐토서혈세포종합정(HPS)적림상특점、진단、치료급림상예후인소。방법회고성분석료26례서혈세포종합정병인、림상특점、실험실검사、치료여전귀。결과발열、림파결종대、간비종대、피부어반、황염시최상견적림상표현。전혈세포감소(100.0%)、혈청철단백승고(61.5%)、혈청유산탈경매증고(96.2%)、곡초전안매/곡병전안매/총담홍소승고(73.1%)、응혈공능이상(65.4%)、감유삼지증다(34.6%)시본조병례실험실특점；골수상：유핵세포증생대다활약,균가견성숙조직세포화서혈세포。16례종류상관성HPS,7례감염상관성HPS급3례원인불명HPS。결론 HPS가유다충원인인기,림상표현복잡다양,림상예후차,응적겁심조원발병인병대환자진행개체화치료,유가능감소병사솔。
Objective To investigate the clinical features, diagnosis, treatment and clinical prognostic factors of hemophagocytic syndrome (HPS). Methods A retrospective study was carried out to analyze the pathogenesis, clinical characteristics, laboratory inspection, treatment and outcomes of 26 HPS cases. Results Common clinical characteristics were fever, lymphadenopathy, splenomegaly, hepatomegaly, petechia and jaundice. Laboratory data features included cytopenia (100.0%), elevated ferritin (61.5%), elevated lactate dehydrogenase (96.2%), increased levels of total bilirubine tbil, aspartate transaminase and alanine aminotransferase (73.1%), coagulation disorder (65.4%), and high level of triglyceride (34.6%). The bone marrow was active, while hemophagocytosis and histiocytes could be seen in bone marrow. There were 16 cases with tumor associated HPS, 7 cases with infection-related HPS, and 3 case with HPS of unknown reason. Conclusion HPS can be caused by complicated reasons, and it has diverse clinical characteristics and poor prognosis. Therefore, finding pathogenesis and providing individualized treatment can possibly reduce the mortality.