Open AccessWaldenström macroglobulinemia
Author(s) -
Klára Baďurová,
Jana Gregorová,
Monika Vlachová,
Marta Krejčí,
Sabina Ševčı́ková
Publication year - 2021
Publication title -
klinická onkologie
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.204
H-Index - 13
eISSN - 1802-5307
pISSN - 0862-495X
DOI - 10.48095/ccko2021428
Subject(s) - waldenstrom macroglobulinemia , macroglobulinemia , hyperviscosity syndrome , medicine , ibrutinib , gammopathy , bone marrow , bruton's tyrosine kinase , monoclonal , cxcr4 , immunology , multiple myeloma , antibody , monoclonal antibody , lymphoma , tyrosine kinase , leukemia , chronic lymphocytic leukemia , receptor , chemokine , immune system
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 aggres-sive disease and patients with this dia-gnosis can live for several years. Infiltration 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 Brutons tyrosine kinase inhibitor, ibrutinib, is most effective in patients with mutated MYD88 and wt CXCR4. The therapy is started when first symptoms occur.