Fukuhara Suguru
Department of Hematology, National Cancer Center Hospital.
Rinsho Ketsueki. 2024;65(9):1012-1018. doi: 10.11406/rinketsu.65.1012.
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.
套细胞淋巴瘤（MCL）是一种在日本较为罕见的淋巴恶性肿瘤。MCL对传统化疗具有耐药性，预后较差。尽管如此，随着自体干细胞移植和BTK抑制剂的出现，MCL的预后已逐渐改善。目前正在研发包含BTK抑制剂的一线治疗方案，有望进一步改善预后。然而，已确定存在预后不良的亚组，包括p53异常（TP53突变或缺失）、母细胞样变异型、高MIPI-c和POD24，这些亚组对包括BTK抑制剂在内的传统治疗均表现出耐药性。为克服这些挑战，诸如CAR-T疗法以及BTK与BCL2抑制剂联合疗法等新型疗法正在研发中，预计不久后将在日本临床应用。MCL的治疗格局正在动态演变，本文将探讨日本MCL治疗策略的未来发展。
