巨球蛋白血症
华登氏巨球蛋白血症
细胞减少
布鲁顿酪氨酸激酶
医学
多发性骨髓瘤
淋巴瘤
基因突变
不确定意义的单克隆抗体病
内科学
伊布替尼
癌症研究
血液学
慢性淋巴细胞白血病
骨髓
单克隆
肿瘤科
免疫学
酪氨酸激酶
白血病
生物
突变
单克隆抗体
基因
抗体
遗传学
受体
标识
DOI:10.3760/cma.j.issn.1009-9921.2019.02.005
摘要
Waldenstrom macroglobulinemia (WM) is a rare type of indolent lymphoma characterized by serum monoclonal IgM. The first symptoms of WM include fever, loss of body mass, cytopenia or organ enlargement. IgM monoclonal gammopathy of undetermined significance (IgM MGUS) and smoldering WM (SWM) are early stages of WM. As the disease is an indolent B-cell lymphoma with low cure rate, the prognosis of the disease has been significantly improved with the in-depth study of its pathogenesis and the development of various new drugs. The MYD88 gene mutation was found to be present in more than 90% of WM patients. MYD88 gene encodes a bridging protein that connects the TLR9 and interleukin (IL)-6R signals to activate the activity of nuclear factor (NF)-κB and mTOR. Ibrutinib is the first Bruton tyrosine kinase inhibitor approved for WM, and it has a significant effect on WM with MYD88 gene mutation. The research on WM reported in the 60th American Society of Hematology (ASH) Annual Meeting explored many aspects. In this paper, the latest research on WM in the 60th ASH Annual Meeting will be reported.
Key words:
Waldenstrom macroglobulinemia; Treatment; Disease progression
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