Waldenströmova makroglobulinemie, klinické příznaky, přehled léčebných možností a vlastní zkušenosti s léčbou ibrutinibem
| Title in English | Waldenström´s macroglobulinemia, overview of clinical symptoms, and therapy and our experience with ibrutinib therapy |
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| Authors | |
| Year of publication | 2022 |
| Type | Article in Periodical |
| Magazine / Source | Onkologie |
| MU Faculty or unit | |
| Citation | |
| Web | https://www.onkologiecs.cz/artkey/xon-202288-0006_waldenstr_mova_makroglobulinemie_klinicke_priznaky_prehled_lecebnych_moznosti_a_vlastni_zkusenosti_s_lecbou_i.php |
| Doi | http://dx.doi.org/10.36290/xon.2022.058 |
| Keywords | Waldenström´s macroglobulinaemia; hyperviscosity; cryoglobulinemia; bendamustin; rituximab; obinutuzumab; ibrutinib |
| Description | Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, in advanced disease hyperviscosity, kryoglobulinemia, B-symptoms and in rare cases the disease can infiltrate CNS (Big-Neel syndrom) or lungs. Presence of IgM monoclonal protein associated with clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in more than 90% of patients and is found in the majority of IgM MGUS patients. Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. Standard therapy is antiCD20 antibody with combination with bendamustine or cyclophospamide and dexamethasone or antiCD20 antibody with a proteosome inhibitor. In patients with early relaps after this therpay we use ibrutinib. Purine nucleoside analogues are active but usage is declining in favor of less toxic alternatives. In the 2022 we prefere for induction rituximab and bendamustine and in case of rituximab intolerance we used obinutuzumab. Given WM‘s natural history, reduction of therapy toxicity is an important part of treatment selection. |