Department of Clinical and Experimental Medicine, School of Medicine, University of Pisa, Pisa, Italy.
UOC Medicina Nucleare, Azienda Ospedaliera Universitaria Sant'Andrea, Rome, Italy.
Chemotherapy. 2024;69(1):40-44. doi: 10.1159/000533236. Epub 2023 Aug 7.
Tuberous sclerosis (TS) is a rare autosomal dominant genetic multisystem disease caused by mutations in either the TSC1 or TSC2 gene and results in the growth of non-cancerous masses in several organs. Diffuse large B-cell lymphoma (DLBCL) is the predominant non-Hodgkin lymphoma in adolescents and young adults. Metronomic chemotherapy (mCHEMO) can be defined as the frequent, regular administration of drug doses able to maintain a low, but active, range of concentrations of chemotherapeutic drugs during prolonged periods of time. We present the case of a young woman with severe TS who developed DLBCL. She was treated consecutively with the mCHEMO schedule R-DEVEC (prednisone, vinorelbine, etoposide, cyclophosphamide, plus rituximab) and then ibrutinib, achieving an impressive long-lasting complete remission. In conclusion, alternative treatments could be necessary when comorbidities are present in patients, and mCHEMO can be a potential successful therapeutic approach in frail subjects.
结节性硬化症（TS）是一种罕见的常染色体显性遗传多系统疾病，由 TSC1 或 TSC2 基因突变引起，导致多个器官内非癌性肿块的生长。弥漫性大 B 细胞淋巴瘤（DLBCL）是青少年和年轻成人中最主要的非霍奇金淋巴瘤。节拍化疗（mCHEMO）可以定义为频繁、有规律地给予药物剂量，以在长时间内维持化疗药物的低但有效的浓度范围。我们报告了一例患有严重 TS 的年轻女性，她患有 DLBCL。她连续接受 mCHEMO 方案 R-DEVEC（泼尼松、长春瑞滨、依托泊苷、环磷酰胺，加利妥昔单抗）和伊布替尼治疗，获得了令人印象深刻的长期完全缓解。总之，当患者存在合并症时，可能需要替代治疗，而 mCHEMO 可能是虚弱患者潜在的成功治疗方法。
