[Update on treatment strategies for mantle cell lymphoma].

Suguru Fukuhara
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Abstract

Mantle cell lymphoma (MCL) is a type of lymphoid malignancy that is rare in Japan. MCL is refractory to conventional chemotherapy and has dismal outcomes. Nonetheless, the prognosis of MCL has gradually improved with the advent of autologous stem cell transplantation and BTK inhibitors. First-line therapies incorporating BTK inhibitors are currently under development, and are expected to further improve the prognosis. Nevertheless, subsets with poor prognosis have been identified, including p53 abnormalities (TP53 mutations or deletions), blastoid variant, high MIPI-c, and POD24, and these show resistance to conventional treatments including BTK inhibitors. To overcome these challenges, novel therapies such as CAR-T therapy and combination therapy with BTK and BCL2 inhibitors are being developed, and should soon become clinically available in Japan. The therapeutic landscape for MCL is evolving dynamically, and this article will discuss the future of MCL treatment strategies in Japan.

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[套细胞淋巴瘤治疗策略的最新进展]。
套细胞淋巴瘤(MCL)是一种淋巴恶性肿瘤,在日本非常罕见。套细胞淋巴瘤对常规化疗难治,疗效不佳。不过,随着自体干细胞移植和BTK抑制剂的出现,MCL的预后逐渐得到改善。含有BTK抑制剂的一线疗法目前正在开发中,有望进一步改善预后。然而,预后不良的亚群已被确定,包括p53异常(TP53突变或缺失)、blastoid变异、高MIPI-c和POD24,这些亚群对包括BTK抑制剂在内的常规治疗表现出耐药性。为了克服这些挑战,目前正在开发 CAR-T 疗法以及 BTK 和 BCL2 抑制剂联合疗法等新型疗法,这些疗法不久将在日本投入临床使用。MCL的治疗格局正在动态演变,本文将讨论日本MCL治疗策略的未来。
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