结节性淋巴细胞为主型霍奇金淋巴瘤：成人淋巴瘤研究组 164 例患者队列的长期研究及转化为弥漫性大 B 细胞淋巴瘤的分析。

Nodular, lymphocyte-predominant Hodgkin lymphoma: a long-term study and analysis of transformation to diffuse large B-cell lymphoma in a cohort of 164 patients from the Adult Lymphoma Study Group.

机构信息

Hematology Department, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.

出版信息

Cancer. 2010 Feb 1;116(3):631-9. doi: 10.1002/cncr.24819.

DOI:10.1002/cncr.24819

PMID:20029973

Abstract

BACKGROUND

Nodular, lymphocyte-predominant Hodgkin lymphoma (NLPHL) represents a rare entity.

METHODS

A clinical registry was launched from 1973 to 2003 in France. To determine the histologic transformation (HT) rate to diffuse large B-cell lymphoma (DLBCL) and long-term outcomes, 164 patients were selected after histologic review.

RESULTS

The median follow-up was 9.5 years. The high biopsy rate (85%) at each recurrence enabled the analysis of HT. The median patient age was 30 years (range, 6-69 years), 80% of patients were men, 83% had Ann Arbor stage I/II disease, 65% had supradiaphragmatic-disease; 27% received radiotherapy, 9% received chemotherapy, 29% received combined-modality therapy, and 35% were followed with a watch-and-wait strategy. All 106 treated patients achieved complete remission and 66 patients developed disease recurrence at a median of 3.3 years (range, 0.4-18.3 years after diagnosis). The majority of recurrences were NLPHL, but 19 patients progressed to DLBCL at a median of 4.7 years (range, 0.4-18 years after diagnosis). The 10-year cumulative HT rate was 12% and was found to be associated significantly with a poor prognosis. The 10-year overall survival rate was 91%. Fourteen patients died (7 died of progressive disease, 3 died of secondary cancers, and 4 died from other causes). HT was diagnosed at a median of 4.7 years (range, 0.4-18 years after diagnosis). The 19 patients who had HT were treated with curative intent: Nine patients received high-dose therapy with subsequent autologous stem cell transplantation (ASCT), and 10 patients received different chemotherapy regimens. The overall survival rate after HT did not differ between patients who underwent ASCT and the others.

CONCLUSIONS

This long-term follow-up study confirmed that NLPHL is a separate entity that has a favorable clinical presentation and outcome despite frequent recurrences. The current findings also emphasize the importance of biopsies at the time patients develop recurrent disease to evaluate HT.

摘要

背景

结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）是一种罕见的疾病。

方法

1973 年至 2003 年，法国启动了一项临床登记研究。为了确定向弥漫性大 B 细胞淋巴瘤（DLBCL）的组织学转化（HT）率和长期结果，在组织学复查后选择了 164 例患者。

结果

中位随访时间为 9.5 年。每次复发时高活检率（85%）使 HT 得以分析。中位患者年龄为 30 岁（范围，6-69 岁），80%的患者为男性，83%的患者为 Ann Arbor 分期 I/II 期疾病，65%的患者为膈上疾病；27%的患者接受了放疗，9%的患者接受了化疗，29%的患者接受了联合治疗，35%的患者接受了观察等待策略。所有 106 例接受治疗的患者均达到完全缓解，66 例患者在中位 3.3 年后（诊断后 0.4-18.3 年）复发。大多数复发为 NLPHL，但 19 例患者在中位 4.7 年后（诊断后 0.4-18 年）进展为 DLBCL。10 年累积 HT 率为 12%，并与不良预后显著相关。10 年总生存率为 91%。14 例患者死亡（7 例死于疾病进展，3 例死于继发性癌症，4 例死于其他原因）。HT 在中位时间 4.7 年后（诊断后 0.4-18 年）被诊断。19 例发生 HT 的患者接受了根治性治疗：9 例患者接受了高剂量治疗随后进行自体干细胞移植（ASCT），10 例患者接受了不同的化疗方案。HT 后总生存率在接受 ASCT 和未接受 ASCT 的患者之间没有差异。