CAJ | 학술논문

目的探讨鼻NK/T细胞淋巴瘤并发噬血细胞综合征(HPS)的临床特征、治疗方法及预后.方法对3例鼻NK/T细胞淋巴瘤并发HPS患者的临床资料进行回顾性分析.结果 3例鼻NK/T细胞淋巴瘤患者符合HPS诊断标准,初诊时具有多项淋巴瘤相关不良预后因素,1例以HPS为首发症状,2例发生HPS时处于疾病进展期,骨髓检查均发现淋巴瘤细胞浸润.并发HPS后患者病情进展迅速,最明显症状是发热、血象进行性下降、纤维蛋白原降低、血清铁蛋白升高及骨髓中出现噬血现象.给予以HLH-2004为基础的方案联合化疗后,HPS均有不同程度改善,但由于原发病无法控制,HPS很快复发,患者并发肝功能异常、凝血异常或弥散性血管内凝血,最终死亡.结论鼻NK/T细胞淋巴瘤并发HPS时预后差,常发生在淋巴瘤进展期或终末阶段.以HLH-2004为基础的方案联合化疗有望逆转病情,延缓疾病进展,为原发病治疗创造机会.
목적 탐토비NK/T세포림파류병발서혈세포종합정(HPS)적림상특정、치료방법급예후.방법 대3례비NK/T세포림파류병발HPS환자적림상자료진행회고성분석.결과 3례비NK/T세포림파류환자부합HPS진단표준,초진시구유다항림파류상관불량예후인소,1례이HPS위수발증상,2례발생HPS시처우질병진전기,골수검사균발현림파류세포침윤.병발HPS후환자병정진전신속,최명현증상시발열、혈상진행성하강、섬유단백원강저、혈청철단백승고급골수중출현서혈현상.급여이HLH-2004위기출적방안연합화료후,HPS균유불동정도개선,단유우원발병무법공제,HPS흔쾌복발,환자병발간공능이상、응혈이상혹미산성혈관내응혈,최종사망.결론 비NK/T세포림파류병발HPS시예후차,상발생재림파류진전기혹종말계단.이HLH-2004위기출적방안연합화료유망역전병정,연완질병진전,위원발병치료창조궤회.
Objective To study clinical features, treatment and prognosis of nasal NK/T cell lymphoma associated Hemophagocytic Syndrome (HPS).Methods Retrospectively analysis method was used to analyze the clinical data of 3 patients with nasal NK/T cell lymphoma associated HPS. Results 3 patients with nasal NK/T cell lymphoma fulfilled the criteria of HPS. All patients had adverse prognostic factors of lymphoma.1 patient developed HPS as the main primary manifestations of underlying lymphoma,the other 2 patients developed HPS during lymphoma progression. In three cases, bone marrow was infiltrated with lymphoma cells.When HPS occurred,the disease progressed rapidly.The most obvious clinical features were fever,pancytopenia,hypofibrinogenemia,hyperferritinemia,and hemophagocytosis in bone marrow. After being treated according to the HLH-2004 combined with chemotherapy, all patients showed a clinical response,but with the progression of lymphoma,HPS quickly relapsed,and all patients died of severe hepatic dysfunction,coagulopathy,or DIC.Conclusion Nasal NK/T lymphoma associated HPS is an invariably fatal disease with poor prognosis,typically occurring at advanced stage or the terminal phase of the disease.HLH-2004 based protocol in combination with chemotherapy is helpful for nasal NK/T cell lymphoma associated HPS,which may delay disease progression and provid opportunities for the treatment of primary disease.