Differential Diagnosis of Waldenstr?m?s Macroglobulinemia and Early Management: Perspectives from Clinical Practice

被引:6
作者
Cingam, Shashank [1 ]
Sidana, Surbhi [2 ,3 ]
机构
[1] Univ New Mexico Comprehens Canc Ctr, Div Hematol & Oncol, Albuquerque, NM 87102 USA
[2] Stanford Univ Sch Med, Div BMT & Cell Therapy, Stanford, CA 94305 USA
[3] Stanford Univ Sch Med, Div Blood & Marrow Transplant & Cellular Therapy, 300 Pasteur Dr Rm H0101c, Stanford, CA 94305 USA
关键词
Waldenstr?m?s Macroglobulinemia; lymphoplasmacytic lymphoma; WM; LPL; MONOCLONAL GAMMOPATHY; RARE COMPLICATION; SINGLE-ARM; OPEN-LABEL; RITUXIMAB; DEXAMETHASONE; MUTATION; THERAPY; MYD88; RISK;
D O I
10.2147/BLCTT.S259860
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Waldenstrom's Macroglobulinemia (WM) is a clonal B-lymphocyte neoplasm characterized by the presence of IgM monoclonal protein and >= 10% bone marrow involvement with lymphoplasmacytic cells. Several mature B-cell and plasma cell disorders can potentially produce monoclonal IgM immunoglobulin and hence, careful consideration of the differential diagnosis is vital. Clinico-pathological features, immunophenotype, and MYD88 mutation status help distinguish WM from other plasma cell and lymphoproliferative disorders. Treatment is only indicated in patients symptomatic from adenopathy or organomegaly, neuropathy, hyper viscosity, cryoglobulinemia, cold agglutinin disease, cytopenia's or amyloidosis. Alkylators (cyclophosphamide, bendamustine) in combination with anti-CD20 antibodies and novel targeted agents including Bruton tyrosine kinase (BTK) inhibitors like ibrutinib are the mainstay of frontline treatment in symptomatic WM.
引用
收藏
页码:107 / 117
页数:11
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