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Patience and commitment needed to fight very rare tumours.
The Lancet Oncology ( IF 41.6 ) Pub Date : 2020-03-11 , DOI: 10.1016/s1470-2045(20)30098-x
Andrés J M Ferreri 1
Affiliation  

Intravascular large B-cell lymphoma (IVLBCL) is a very rare and aggressive neoplasm characterised by selective growth of tumour cells within blood vessel lumina. This peculiar growth pattern, the virtual absence of tumour masses, and the multifaceted and severe presentation make IVLBCL an attractive study model, but published work on this disease is scarce and fragmentary. More than a decade ago, international researchers established diagnostic and therapeutic recommendations for IVLBCL and distinguished two clinical forms: classic IVLBCL, which affects almost all patients diagnosed in European and North American countries; and a haemophagocytic syndrome-related form, which is a more aggressive type and is frequently diagnosed in Japan. After two studies from the Japanese Society of Hematology and the International Extranodal Lymphoma Study Group showed a positive survival effect of rituximab, most patients with IVLBCL have been treated with R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone). Subsequent studies have shown that the CNS is the main site of recurrence after treatment with R-CHOP, providing the rationale for addition of a CNS-oriented strategy to treatment regimens.

中文翻译:

抵抗非常罕见的肿瘤需要耐心和奉献精神。

血管内大B细胞淋巴瘤(IVLBCL)是一种非常罕见的侵袭性肿瘤,其特征是肿瘤细胞在血管腔内选择性生长。这种独特的生长方式,几乎没有肿瘤块以及多方面且严重的表现使IVLBCL成为有吸引力的研究模型,但是有关该病的已发表研究很少而且还很少。十多年前,国际研究人员建立了IVLBCL的诊断和治疗建议,并区分了两种临床形式:经典的IVLBCL,它影响几乎所有在欧洲和北美国家诊断出的患者;以及与噬血细胞综合征相关的形式,这是一种更具侵略性的形式,在日本经常被诊断出来。经过日本血液学会和国际淋巴结外淋巴瘤研究小组的两项研究表明,利妥昔单抗具有积极的生存作用,大多数IVLBCL患者已接受R-CHOP治疗(利妥昔单抗加环磷酰胺,阿霉素,长春新碱和泼尼松)。随后的研究表明,CNS是R-CHOP治疗后复发的主要部位,, 为在治疗方案中添加面向中枢神经系统的策略提供了理论依据。
更新日期:2020-03-31
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