Waldenström macroglobulinemia

Abstract

Background: Waldenström macroglobulinemia (WM) is a hematological malignancy; it is a monoclonal gammopathy, a disease characterized by presence of a monoclonal immunoglobulin in serum and/ or urine. The median age at dia gnosis is 71 years. WM is not an aggressive disease and patients with this dia gnosis can live for several years. Infi ltration of the bone marrow with lymphoplasmacytoid cells causes anemia, leading to various problems, mainly fatigue. Hepatomegaly, splenomegaly and lymphadenopathy can also occur. Hyperviscosity syndrome can appear and is caused by excessive production of immunoglobulin M. A mutation in MYD88 gene is detected in almost every WM patient, and in almost one third of them, a mutation in CXCR4 gene is detected. The detection of MYD88 mutation is important for a correct therapeutic strategy, since a Bruton’s tyrosine kinase inhibitor, ibrutinib, is most eff ective in patients with mutated MYD88 and wt CXCR4. The therapy is started when fi rst symptoms occur. Purpose: The aim of this study is to summarize current knowledge about this disease, its dia gnostics, molecular basis and treatment.