Biology, clinical course and management of nodular lymphocyte-predominant hodgkin lymphoma.
Biology, clinical course and management of nodular lymphocyte-predominant hodgkin lymphoma.
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DOI：
10.1182/asheducation-2006.1.266
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发表时间：
2006-01-01
期刊：
影响因子：
--
通讯作者：
Engert, Andreas
中科院分区：
文献类型：
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作者：
Nogova, Lucia;Rudiger, Thomas;Engert, Andreas
以结节淋巴细胞为主的霍奇金淋巴瘤(NLPHL)在组织学和临床表现上不同于经典型霍奇金淋巴瘤(CHL)。典型的非淋巴细胞性淋巴瘤的形态特征是不典型的“淋巴细胞性和组织细胞性”(L和H)细胞，周围有非肿瘤性结节背景的B细胞起源的小淋巴细胞。NLPHL细胞表达CD45、CD19、CD20、CD22和CD79a，但不表达典型的CHL标志物CD15和CD30。NLPHL患者主要为男性，平均年龄37岁。患者通常出现在早期(63%)，很少有B-症状(9%)。使用标准霍奇金淋巴瘤(HL)方案治疗NLPHL患者，95%以上的患者完全缓解(CR)。晚期患者的存活率和免于治疗失败(FFTF)比早期患者更差。因此，具有不利危险因素的晚期和早期患者的治疗方式与慢性粒细胞白血病患者相似。相比之下，没有危险因素的早期NLPHL患者可以通过降低强度的计划进行充分的治疗，其不良反应较轻。因此，早期NLPHL的治疗还不是很明确，包括扩展野(EF)或累及野(IF)放射治疗技术，综合治疗方法，以及最近的单抗利妥昔单抗。观察和等待策略在儿科肿瘤学中起着重要的作用，以避免与治疗相关的不良反应。
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) differs in histological and clinical presentation from classical Hodgkin lymphoma (cHL). The typical morphologic signs of NLPHL are atypical "lymphocytic and histiocytic" (L&H) cells, which are surrounded by a non-neoplastic nodular background of small lymphocytes of B-cell origin. The NLPHL cells are positive for CD45, CD19, CD20, CD22 and CD79a, but lack expression of CD15 and CD30, the typical markers for cHL. NLPHL patients are predominantly of male gender with a median age of 37 years. Patients often present in early stages (63%) and rarely have B-symptoms (9%). Treatment of NLPHL patients using standard Hodgkin lymphoma (HL) protocols leads to complete remission (CR) in more than 95% of patients. Survival and freedom from treatment failure (FFTF) are worse in advanced-stage patients than in early-stage patients. Thus, patients in advanced and in early stages with unfavorable risk factors are treated similarly to cHL patients. In contrast, patients with early-stage NLPHL without risk factors can be sufficiently treated with reduced intensity programs having less severe adverse effects. As a result, treatment of early NLPHL is less clearly defined, including radiotherapy in extended field (EF) or involved field (IF) technique, combined modality treatment, and, more recently, monoclonal antibody rituximab. Watch and wait strategy plays an important role in pediatric oncology to avoid adverse effects associated with therapy.