ObjectiveTo improve the understanding, diagnosis and treatment of special clinical characteristics of Waldenstrom macroglobulinemia (WM). MethodsWe described one case of WM which initially presented with jaundice, and discussed the clinical characteristics, diagnosis and therapeutics based on the literature review. ResultsWM is a B-cell lymphoproliferative disorder characterized by an immunoglobulin M (IgM) monoclonal gammopathy and bone marrow infiltration by lymphoplasmacytic lymphoma, which generally affects elderly patients. WM with primary jaundice is rare in clinic, which is usually misdiagnosed and has missed diagnosis. Patients should have their IgM monitored first before accepting therapeutic regimen including rituximab in order to avoid IgM flare or related complications. ConclusionsThe potential diagnosis of malignant plasma cell disorder should be considered for those patients who presented with jaundice initially, after liver system diseases are excluded. Liver biopsy and related examinations are necessary without delay. Prophylactic plasmapheresis is an active means to lower the incidence of IgM flare, especially for patients with a pre-therapy IgM level of higher than 50 g/L.
Waldenström’s macroglobulinemia (WM)/lymphoplasmacytic lymphoma (LPL) is a rare mature B-cell neoplasm. WM is characterized by the presence of a lymphoplasmacytic infiltrate in the bone marrow and high serum levels of monoclonal immunoglobulin M protein. With a deeper understanding of molecular mechanisms of the disease, the update of diagnostic approaches and the introduction of novel therapies, the management of WM/LPL has rapidly evolved over the past few years. On March 7th, 2018, National Comprehensive Cancer Network (NCCN) updated the guideline for WM/LPL. This study mainly interpreted the corresponding diagnosis and treatment of WM/LPL in NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®): Waldenström’s Macroglobulinemia/Lymphoplasmacytic Lymphoma (Version 1.2018).
Waldenström macroglobulinemia (WM) is a rare indolent lymphoplasmacytic lymphoma. Bruton’s tyrosine kinase (BTK) plays a key role in the signaling pathway of WM, which has changed the way of treatment of WM. As a first-generation BTK inhibitor, ibrutinib is an early-stage treatment and a salvage treatment that can control toxicity characteristics. However, in order to overcome the resistance of the first-generation BTK inhibitors, reduce the adverse reactions caused by off-target effects, and improve the efficacy, the research and development of new BTK inhibitors has become a hot topic. This article discusses the clinical studies of the first generation and new BTK inhibitors for WM, aiming to provide a certain basis for the more rational application of BTK inhibitors in WM.