摘要目的:探讨以噬血细胞性淋巴组织细胞增多症(HLH)为首发表现的非霍奇金淋巴瘤(NHL)患者的临床特征及预后，并且进行相关文献复习。方法:选择2018年10月至2021年1月，于黑龙江省医院收治的5例以HLH为首发表现的NHL患者为研究对象，并根据患者入院时间，将其依次编号为患者1~5。患者年龄为63~81岁；男性患者为2例，女性为3例。采用回顾性研究方法，收集本组患者的临床病例资料，包括临床特征和HLH相关实验室检查结果、治疗方案及预后等。并以"噬血细胞综合征""噬血细胞性淋巴组织细胞增生症""淋巴瘤""首发""hemophagocytic syndrome""hemophagocytic lymphohistiocytosis""lymphoma""first manifestation"为中、英文关键词，在中国知网(CNKI)数据库、万方数据知识服务平台及PubMed数据库中，检索以HLH为首发表现NHL相关文献，并且进行文献复习。本研究遵循的程序符合2013年修订的《世界医学协会赫尔辛基宣言》要求。结果:①临床特征：5例患者均继发于NHL，其中T细胞NHL(T-NHL)和B细胞NHL(B-NHL)分别为3和2例；均以持续发热就诊，并伴淋巴结和脾大，2例伴肝大。②实验室检查结果：5例患者均伴血细胞减少和骨髓噬血现象，以及sCD25水平升高；血清铁蛋白(SF)水平>500 μg/L者为3例，高甘油三酯血症(HTG)和自然杀伤(NK)细胞活性减低者各为2例，低纤维蛋白原血症者仅为1例。③治疗及转归：3例T-NHL患者未接受系统治疗，均于1个月内死亡；2例B-NHL患者分别经地塞米松(Dex)+依托泊苷(VP16)或R-CHOPE(利妥昔单抗+环磷酰胺+多柔比星+长春新碱+地塞米松+依托泊苷)方案和Dex+VP16或COPE(环磷酰胺+长春新碱+地塞米松+依托泊苷)方案治疗后，达到完全缓解(CR)和部分缓解(PR)。④文献复习结果：根据本研究文献检索策略及筛选标准，共计纳入3篇相关文献，涉及4例以HLH为首发表现的NHL患者，其中弥漫大B细胞淋巴瘤(DLBCL)患者为2例，B-NHL为1例，NK/T细胞淋巴瘤(NKTCL)为1例。3例B-NHL患者主要接受Dex+VP16或R-CHOP±E(利妥昔单抗+环磷酰胺+多柔比星+长春新碱+地塞米松±依托泊苷)方案治疗后，2例DLBCL患者获得CR，1例B-NHL患者达到PR。1例NKTCL患者经多种方案治疗后，疗效不佳，最终死亡。结论:以HLH为首发表现的NHL患者通常预后较差。其中，B-NHL患者主要以R-CHOP±E方案治疗，预后相对较好。

abstractsObjective:To explore clinical features and prognosis of hemophagocytic lymphohistiocytosis (HLH) as the first manifestation in patients with non-Hodgkin lymphoma (NHL), and to review relevant literature.Methods:From October 2018 to January 2021, a total of 5 cases of NHL patients with HLH as the first manifestation admitted to Heilongjiang Provincial Hospital were selected as study subjects, and then numbered as patient 1 to 5 according to their admission time. Patients′ ages ranged from 63 to 81 years. There were 2 male and 3 female patients. A retrospective study was conducted to collect clinical data of these patients, including clinical features, HLH-related laboratory test results, treatment and prognosis. With " hemophagocyticocytosis syndrome" " hemophagocytic lymphohistiocytosis" " lymphoma" and " first manifestation" as Chinese and English keywords, relevant literature on NHL patients with HLH as the first manifestation were searched in the China National Knowledge Infrastructure (CNKI)database, Wanfang Data Knowledge Service Platform and PubMed database. And relevant literature was reviewed. The procedures followed in this study were in line with the requirements of World Medical Association Declaration of Helsinki revised in 2013. Results:① The clinical characteristics of these 5 patients were as follows: all 5 patients were secondary to NHL, including 3 patients with T-cell NHL (T-NHL) and 2 cases with B-cell NHL (B-NHL). All patients presented with persistent fever, lymphadenopathy and splenomegaly. And 2 of them had been associated with hepatomegaly. ② The results of laboratory test were as follows: all 5 patients had cytopenias and bone marrow hemophagocytosis, and increased serum sCD25 levels. There were 3 patients with serum ferritin (SF) levels higher than 500 μg/L, and 2 patients with reduced activity of natural killer (NK) cell. Hypertriglyceridemia was recorded in 2 patients, while only 1 patient was associated with hypofibrinogenemia. ③ Treatment and outcomes of patients were as follows: three T-NHL patients without systemic treatment died within 1 month. By contrast, 2 B-NHL cases were achieved complete remission (CR) and partial remission (PR), after treated with dexamethasone (Dex)+ etoposide (VP16) or R-CHOPE (rituximab+ cyclophosphamide+ doxorubicin+ vincristine+ prednisolone+ etoposide) and Dex+ VP16 or COPE (cyclophosphamide+ vincristine+ dexamethasone+ etoposide) regimens, respectively. ④ According to the literature search and screening strategies of this study, a total of 3 related literature were included, involving 4 NHL patients with HLH as the first manifestation. Among them, there were 2 patients with diffuse large B cell lymphoma (DLBCL), 1 case with B-NHL and 1 case with NK/T-cell lymphoma (NKTCL). After treated with Dex+ VP16 or R-CHOP±E (rituximab+ cyclophosphamide+ doxorubicin+ vincristine+ prednisolone±etoposide), 2 DLBCL patients achieved CR and 1 B-NHL patient achieved PR. One patient with NKTCL eventually died after multiple regimen treatments.Conclusions:NHL patients with HLH as the first manifestation have a poor prognosis. Among them, B-NHL patients who were treated with R-CHOP±E regimen showed a relatively good prognosis.