Clinical characteristics of extranodal NK/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis
Clinical characteristics of extranodal NK/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis
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DOI：
10.2147/cmar.s183784
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发表时间：
2019-01-01
影响因子：
3.3
通讯作者：
Sun, Si
中科院分区：
文献类型：
--
作者：
Liu, Yi-Zhen;Bi, Lan-Qing;Sun, Si
背景：结外自然杀伤(NK)/T细胞淋巴瘤是非霍奇金淋巴瘤(NHL)的一个亚型，具有侵袭性的临床病程。它是淋巴瘤相关噬血细胞综合征(LAHS)的主要诱因，这种综合征具有高度的致命性和极差的预后。本研究旨在探讨结外NK/T细胞淋巴瘤相关性噬血细胞组织细胞增生症的临床特点及预后因素。结果：最常见的临床特征为发热(96.4%)和脾肿大(81.5%)。实验室检查以高铁蛋白血症(91.7%)、III/IV级血小板减少症(64.3%)、高甘油三酯血症(48%)、重度贫血(46.4%)、低纤维蛋白原血症(45%)和III/IV级中性粒细胞减少症(32.1%)最常见。确诊至死亡/最后一次接触的时间为4~701天，中位数为15天。我们发现HLH值越高、低纤维蛋白原血症和脾肿大患者的生存率越差(P=0.002、0.003、0.003)。此外，东部合作肿瘤组(ECOG)评分、上呼吸道外NK/T细胞淋巴瘤(EUNKTL)和皮肤受累是HLH的危险因素。结论：LDH、纤维蛋白原和脾肿大是影响HLH预后的因素。较高的ECOG评分、EUNKTL和皮肤受累是NK/T LAHS的危险因素。将需要更多的独立的、前瞻性的临床试验来探索最佳治疗方法。
Background: Extranodal natural killer (NK) / T cell lymphoma is a subtype of non-Hodgkin's lymphoma (NHL) that usually has an aggressive clinical course. It is the predominant trigger of lymphoma-associated hemophagocytic syndrome (LAHS), which is highly lethal and with extremely poor prognosis. This study is aiming to characterize the associated clinical features and prognostic factors of the disease.Patients and methods: Twenty-eight patients with extranodal NK/T cell lymphoma associated hemophagocytic lymphohistiocytosis (HLH) were retrospectively analyzed. The clinical records were collected, and the associations between clinical or laboratory parameters and overall survival (OS) were assessed.Results: The most frequently clinical characteristics were fever (96.4%), and splenomegaly (81.5%). Concerning the laboratory findings, the most common features were hyperferritinemia (91.7%), grade III/IV thrombocytopenia (64.3%), hypertriglyceridemia (48%), severe anemia (46.4%), hypofibrinogenemia (45%), and grade III/IV neutropenia (32.1%). The interval between the diagnosis of NK/T LAHS and death /last contact was between 4 to 701 days with the median interval of 15 days. We found that higher serum lactic dehydrogenase (LDH) at HLH, hypofibrinogenemia, and splenomegaly were significantly associated with worse survival (P=0.002, 0.003, 0.003). Furthermore, Eastern Cooperative Oncology Group (ECOG) score, extra-upper aerodigestive tract NK/T cell lymphoma (EUNKTL) and cutaneous involvement were risk factors of HLH.Conclusion: Our data indicated that levels of LDH, fibrinogen, and presence of splenomegaly were prognostic factors of the disease. Higher ECOG scores, EUNKTL and cutaneous involvement were risk factors of NK/T LAHS. Additional independent, prospective clinical trials will be needed to explore optimal treatment.