Department of Pathology, Cancer Center Amsterdam, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.
Lymphoma and Myeloma Center Amsterdam - LYMMCARE, Amsterdam, The Netherlands.
Leukemia. 2021 Mar;35(3):881-886. doi: 10.1038/s41375-020-0934-6. Epub 2020 Jun 26.
Mantle cell lymphoma (MCL) is an aggressive non-Hodgkin lymphoma subtype arising from naïve B cells. Although novel therapeutics have improved patient prognosis, drug resistance remains a key problem. Here, we show that the SRC-family tyrosine kinase hematopoietic cell kinase (HCK), which is primarily expressed in the hematopoietic lineage but not in mature B cells, is aberrantly expressed in MCL, and that high expression of HCK is associated with inferior prognosis of MCL patients. HCK expression is controlled by the toll-like receptor (TLR) adaptor protein MYD88 and can be enhanced by TLR agonists in MCL cell lines and primary MCL. In line with this, primary MCL with high HCK expression are enriched for a TLR-signaling pathway gene set. Silencing of HCK expression results in cell cycle arrest and apoptosis. Furthermore, HCK controls integrin-mediated adhesion of MCL cells to extracellular matrix and stromal cells. Taken together, our data indicate that TLR/MYD88-controlled aberrant expression of HCK plays a critical role in MCL proliferation and survival as well as in retention of the malignant cells in the growth- and survival-supporting lymphoid organ microenvironment, thereby contributing to lymphomagenesis. These novel insights provide a strong rationale for therapeutic targeting of HCK in MCL.
套细胞淋巴瘤(MCL)是一种起源于幼稚 B 细胞的侵袭性非霍奇金淋巴瘤亚型。尽管新型治疗方法改善了患者的预后,但耐药性仍然是一个关键问题。在这里,我们表明,主要在造血谱系中表达但不在成熟 B 细胞中表达的 Src 家族酪氨酸激酶造血细胞激酶(HCK)在 MCL 中异常表达,并且 HCK 高表达与 MCL 患者的预后不良相关。HCK 表达受 Toll 样受体(TLR)衔接蛋白 MYD88 控制,TLR 激动剂可增强 MCL 细胞系和原代 MCL 中的 HCK 表达。与此一致的是,HCK 高表达的原发性 MCL 富含 TLR 信号通路基因集。沉默 HCK 表达会导致细胞周期停滞和细胞凋亡。此外,HCK 控制 MCL 细胞与细胞外基质和基质细胞的整合素介导的黏附。总之,我们的数据表明,TLR/MYD88 控制的 HCK 异常表达在 MCL 的增殖和存活中以及在恶性细胞在支持生长和存活的淋巴器官微环境中的保留中发挥关键作用,从而促进淋巴瘤的发生。这些新的见解为在 MCL 中靶向 HCK 治疗提供了强有力的理由。