Progress of Waldenstrom macroglobulinemia / 白血病·淋巴瘤

ABSTRACT

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.