Diagnostika a léčba Waldenströmovy makroglobulinemie

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Title in English Diagnosis and treatment of Waldenström's macroglobulinemia
Authors

ADAM Zdeněk HÁJEK Roman KREJČÍ Marta MAISNAR Vladimír ŘÍHOVÁ Lucie ŠEVČÍKOVÁ Sabina BELADA David KAŠČÁK Michal TRNĚNÝ Marek

Year of publication 2014
Type Article in Periodical
Magazine / Source Transfuze a hematologie dnes
MU Faculty or unit

Faculty of Medicine

Citation
Field Oncology and hematology
Keywords bendamustin; bortezomib; rituximab; hyperviscosity; cryoglobulinemia; cold agglutinin disease; monoclonal imunoglobulin related disorders; Waldenström's macroglobulinaemia
Description Therapy of Waldenström's macroglobulinaemia (WM) is indicated in patients with clinically relevant symptoms. Therapeutic plasmapheresis should be performed in cases with hyperviscosity. The intensity of chemotherapy should be adjusted to the degree of cytopenia. Monotherapy with rituximab is recommended in cases with severe cytopenia, also combination of rituximab with dexamethasone should be possible. Patients with symptomatic WM without severe cytopenia should received a rituximab-containing regimens, optimal variant is combination of rituximab + dexamethasone + alkylation drug (such as cyclophosphamide or bendamustine). Possible treatment combinations are for instance R-CHOP (rituximab, cyclophosphamide, vincristine, and prednisone), R-COP (rituximab, cyclophosphamide, and prednisone), or RCD (rituximab, cyclophosphamide, and dexamethasone). The choice of regimen in individual patients will take into consideration performance status, clinical features including renal function, comorbidities and potential candidacy for stem cell transplantation. Chlorambucil and rituximab is possible treatment options for older patients. The choice of treatment of WM relapse depends on the time of treatment response. Retreatment with primary therapy may be appropriate in patients with duration of treatment response at least 2 years. Other treatment possibilities for WM relapse are regimens containing fludarabine, cladribine or bortezomib. Autologous transplantation of peripheral blood stem cells is feasible therapeutic option for relapsed WM in younger, fitter patients with aggressive chemosensitive disease.
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