Department of Haematology, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre Bénite cedex, France.
INSERM Unité Mixte de Recherche (UMR)-S1052, Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, Lyon, France-Equipe Labellisée Ligue Contre le Cancer - Université Claude Bernard Lyon 1, Pierre Bénite cedex, France.
Br J Haematol. 2020 Apr;189(2):244-256. doi: 10.1111/bjh.16331. Epub 2020 Feb 6.
Composite and sequential lymphomas involving both classical Hodgkin lymphoma (CHL) and primary mediastinal B-cell lymphoma (PMBCL) are rare phenomena. Beyond the relevant biological interest raised by these cases, treatments and outcome data are poorly covered in the recent literature. This retrospective analysis describes the pathological and clinical characteristics of 10 composite and 15 sequential cases included after a central pathological review. At diagnosis, 70% of the composite lymphomas presented a disseminated and extranodal disease. Among the 15 sequential lymphomas, 12 were CHL at first occurrence and three were PMBCL. Based on their clinical evolution, these sequential lymphomas could be divided into early (i.e., diagnosis of second lymphoma within a year) and late [(i.e., a second lymphoma occurrence occurring after a long period of complete remission]). All composite cases were alive in complete remission after a median follow-up of 34 months. If the early sequential lymphoma presented a particularly poor outcome with a median overall survival shorter than one year, the late cases were efficiently salvaged. Further molecular studies are needed to describe the underlying biology of these rare diseases, possibly representing the extreme of tumour cell plasticity found in grey-zone lymphoma.
涉及经典霍奇金淋巴瘤(CHL)和原发性纵隔 B 细胞淋巴瘤(PMBCL)的复合和序贯淋巴瘤是罕见现象。除了这些病例引起的相关生物学兴趣外,最近的文献中对这些病例的治疗和预后数据报道较少。本回顾性分析描述了经过中心病理复查后纳入的 10 例复合和 15 例序贯病例的病理和临床特征。在诊断时,70%的复合淋巴瘤表现为播散性和结外疾病。在 15 例序贯性淋巴瘤中,12 例首先发生 CHL,3 例首先发生 PMBCL。根据其临床演变,这些序贯性淋巴瘤可分为早期(即第二个淋巴瘤在一年内诊断)和晚期(即第二个淋巴瘤在长时间完全缓解后发生)。所有复合病例在中位随访 34 个月后均处于完全缓解且存活。如果早期序贯性淋巴瘤的总生存中位数短于 1 年,预后特别差,则晚期病例可被有效挽救。需要进一步的分子研究来描述这些罕见疾病的潜在生物学,这些疾病可能代表了灰色区域淋巴瘤中肿瘤细胞可塑性的极端表现。