目的:分析淋巴瘤相关噬血细胞综合征(lymphoma-associated hemophagocytic lymphohistiocytosis,LA-HLH)的临床特征,探讨不同治疗方案对预后的影响.方法:回顾性分析2021年7月至2023年7月在我院血液科确诊的32例LA-HLH患者的临床资料,比较不同淋巴瘤病理亚型LA-HLH患者的临床特征和预后差异.结果:纳入的32例患者均符合HLH-2004的诊断标准,经组织病理确诊为淋巴瘤且年龄大于18岁.①临床特征:32例患者中位年龄58岁(范围:18～82岁),17例(53.1％)为男性;中位诊断时间11.5 d(范围:2～56 d);病理类型分别为B细胞非霍奇金淋巴瘤(B-NHL)15例、T/NK细胞非霍奇金淋巴瘤(T/NK-NHL)15例和经典霍奇金淋巴瘤2例.所有患者均有发热和铁蛋白升高;发生比例比较高的其他临床特征包括乳酸脱氢酶升高(96.9％)、淋巴细胞减少(90.6％)、≥2系的血细胞减少(87.5％)、脾大(84.4％)、低蛋白血症(84.4％)、sCD25水平升高(75.0％)、肝功能不全(68.8％)、噬血现象(65.6％)和黄疸(62.5％).32例患者中位Hscore评分为250.5(范围:178～376),均高于截断值169.②32例患者中位总生存时间(OS)207 d.与T/NK-NHL患者的中位OS89d比较,B-NHL患者的中位OS(未达到)有延长的趋势,但差异无统计学意义(P＞0.05).在接受HLH诱导治疗的患者中,接受含依托泊苷方案治疗的患者较仅接受地塞米松治疗的患者中位OS明显延长(335 d vs 7d,P＜0.01).此外,接受淋巴瘤化疗方案的患者较仅接受HLH诱导治疗方案的患者中位OS明显延长(未达到vs 23 d,P＜0.01).③15例合并EBV感染的LA-HLH患者均为EBV相关淋巴瘤,其中T/NK细胞淋巴瘤的EBV感染率为86.7％,B细胞淋巴瘤的EBV感染率为6.7％.结论:在诱发HLH的淋巴瘤类型中,B-NHL和T/NK-NHL所占的比例相当,远高于霍奇金淋巴瘤,最常见诱发HLH的3种淋巴瘤类型分别为弥漫大B细胞淋巴瘤、结外NK/T细胞淋巴瘤和血管免疫母细胞淋巴瘤.应用Hscore评分或能帮助临床早期识别LA-HLH患者.基于依托泊苷的HLH诱导治疗序贯多药联合化疗治疗淋巴瘤是LA-HLH治疗的合理选择.
Clinical characteristics and prognosis of adult lymphoma-associated hemophagocytic lymphohistiocytosis
Objective:To examine the clinical characteristics of lymphoma-associated hemophagocytic lympho-histiocytosis(LA-HLH)and investigate the effects of various treatment strategies on the prognosis of patients.Methods:The clinical data of 32 LA-HLH patients diagnosed from July 2021 to July 2023 were retrospectively an-alyzed.The clinical characteristics and prognosis differences among patients with different lymphoma pathological subtypes were compared.Results:All 32 patients met the HLH-2004 criteria,were pathologically diagnosed as malignant lymphomas,and were older than 18 years.The clinical characteristics were as follows:①The median age of 32 patients was 58 years(range:18-82 years),with 17 cases(53.1％)were male.The median diagnostic time was 11.5 days(range:2-56 days).The pathological types were B-cell non-Hodgkin's lymphoma(B-NHL)(15 cases),T/NK-cell non-Hodgkin's lymphoma(T/NK-NHL)(15 cases),and classic Hodgkin's lymphoma(2 ca-ses).All patients had fever and increased ferritin.The other common clinical manifestations included elevated lac-tic dehydrogenase(96.9％),lymphocytopenia(90.6％),cytopenias affecting≥2 of 3 lineages in the peripheral blood(87.5％),splenomegaly(84.4％),hypoproteinemia(84.4％),elevated soluble CD25 level(75.0％),liver dysfunction(68.8％),hemophagocytosis(65.6％),and jaundice(62.5％).The median Hscore value of 32 pa-tients was 250.5(range:178-376),all of which were higher than the cut-off value of 169.②The median overall survival(OS)of 32 patients was 207 days.Compared with the median OS of 89 days in the T/NK-NHL group,the median OS of the B-NHL group(not reached)showed a trend of prolongation,but there was no significant difference(P＞0.05).Among the patients receiving HLH induction therapy,those receiving etoposide-containing regimen had a significantly longer median OS than those receiving dexamethasone alone(335 d vs 7 d,P＜0.01).In addition,patients receiving lymphoma chemotherapy regimen had a significantly longer median OS than those receiving HLH induction therapy only(not reached vs 23 d,P＜0.01).③Among the 15 LA-HLH patients with concurrent EBV infection,all were diagnosed as EBV-related lymphomas,and the EBV infection rate was 86.7％in T/NK cell lymphomas and 6.7％in B cell lymphomas.Conclusion:The proportion of B-NHL and T/NK-NHL is relatively equal in LA-HLH,and significantly higher than that of Hodgkin's lymphoma.The three most com-mon types of lymphomas that induce HLH are diffuse large B-cell lymphoma,extranodal T/NK-cell lymphoma,and angio-immunoblastic T-cell lymphoma.The application of the HScore may aid in the early clinical identifica-tion of patients with LA-HLH.An etoposide-containing HLH induction therapy followed by multidrug combined chemotherapy is a reasonable choice for the treatment of LA-HLH.
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