Onkológia 1/2019
Waldenström‘s macroglobulinemia and treatment
Waldenström‘s macroglobulinemia (WM) is a rare indolent malignant lymphoproliferative disease of mature B-lymphocytes with secretory activity causes monoclonal gammopathy of IgM in serum, and accumulation of these cells in the bone marrow produce a lymphoplasmocytic lymphoma (LPL) picture. Both of these findings are responsible for the symptoms of the disease, but other organs and lymph nodes may also be affected. Accumulation of paraprotein IgM changes the rheological properties of the blood, which are the cause of symptoms of the hyperviscosity syndrome. In the case of symptomatic (on disease) or expected („flare“ effect of rituximab) hyperviscosity, plasmapheresis is a rapid and effective first treatment step. This is followed by systemic first-line treatment based on rituximab, as monotherapy or in combination with cyclophosphamide, bendamustine, bortezomib, glucocorticoids, according to the MYD88 mutation with ibrutinib, which can also be used alone. For a selected group of patients consolidation may be associated with autologous hematopoietic stem cell transplantation, which is also considered for relapsed or refractory WM. In the early relapse within 24 months, we choose an alternative regimen that contains drugs from a different group than those used as primary therapy, e.g. new molecules. Efficacy in WM, with a different spectrum of adverse effects, was also confirmed by ofatumumab, carfilzomib, cladribine, fludarabine, everolimus, thalidomide, lenalidomide, and alemtuzumab. Patients with refractory or relapsed WM are suitable candidates for treatment in the context of available clinical trials
Keywords: Waldenström's macroglobulinemia, plasmapheresis, rituximab, bendamustine, ibrutinib, bortezomib
