Nodular lymphocyte-predominant Hodgkin lymphoma: a unique disease deserving unique management
Nodular lymphocyte-predominant Hodgkin lymphoma: a unique disease deserving unique management
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DOI：
10.1182/asheducation-2017.1.324
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发表时间：
2017-12-01
影响因子：
3
通讯作者：
Engert, Andreas
中科院分区：
文献类型：
--
作者：
Eichenauer, Dennis A.;Engert, Andreas
结节淋巴细胞占优势的霍奇金淋巴瘤是一种罕见的淋巴瘤，发病率为0.10~0.20/100000。与更常见的典型霍奇金淋巴瘤亚型相比，该淋巴瘤具有明显的病理和临床特征。组织学上，定义疾病的淋巴细胞优势细胞持续表达CD20，但缺乏CD30。临床上，NLPHL的病程大多相当缓慢，患者通常在早期被诊断出来。早期NLPHL预后良好，单纯介入野放射治疗(IA期)或联合化疗加2个周期的ABVD(阿霉素、新霉素、长春新碱、达卡巴津)化疗后IF-RT(IA期除外)化疗后无进展生存，总生存率超过90%。相比之下，确诊时患有晚期疾病的患者倾向于复发，无论是NLPHL组织学还是组织学转化为侵袭性B细胞非霍奇金淋巴瘤，尽管有更积极的一线治疗和6至8个周期的多药化疗。然而，即使是多次复发的NLPHL患者，在许多情况下也对抢救治疗有成功的反应。挽救治疗的范围从单剂抗CD20抗体治疗到大剂量化疗后再进行自体干细胞移植。疾病复发的治疗应该根据不同的因素来选择，包括复发的组织学、复发的时间、复发的疾病程度和先前的治疗。由于NLPHL患者的死亡更多地是由治疗相关的后遗症引起的，而不是与淋巴瘤相关的并发症，因此尽可能减少毒性来优化治疗的风险-收益比是这种疾病临床研究的主要目标。
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma entity with an incidence of 0.1 to 0.2/100000/y.Compared with the more common subtypes of classical Hodgkin lymphoma, NLPHL is characterized by distinct pathological and clinical features. Histologically, the disease-defining lymphocyte predominant cells consistently express CD20 but lack CD30. Clinically, NLPHL mostly has a rather indolent course, and patients usually are diagnosed in early stages. The prognosis of early-stage NLPHL is excellent, with progression-free survivaI and overall survival rates exceeding 90% after involved-field radiotherapy (IF-RT) alone (stage IA) or combined modality treatment consisting of a brief chemotherapy with 2 cycles of ABVD (doxorubicin, Neomycin, vinblastine, dacarbazine) chemo therapy followed by IF-RT (early stages other than stage IA). In contrast, patients with advanced disease at diagnosis tend to relapse either with NLPHL histology or with histological transformation into aggressive B-cell non-Hodgkin lymphoma despite more aggressive first-line treatment with 6 to 8 cycles of multiagent chemotherapy. However, even NLPHL patients with multiple relapses successfully respond to salvage therapy in many cases. Salvage therapies range from single-agent anti-CD20 antibody treatment to high-dose chemotherapy followed by autologous stem cell transplantation. Treatment at disease recurrence should be chosen on the basis of various factors, including histology at relapse, time to relapse, extent of disease at relapse, and prior treatment. Because death among NLPHL patients is more often caused by therapy-related late effects than lymphoma-related complications, optimizing the risk-benefit ratio of treatment by decreasing toxicity whenever possible is the major goal of clinical research in this disease.