Změny v prognóze a v léčbě Waldenströmovy makroglobulinemie: přehled literatury a vlastní zkušenosti
| Title in English | Changes in the prognosis and treatment of Waldenström macroglobulinemia. Literature overview and own experience |
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| Authors | |
| Year of publication | 2016 |
| Type | Article in Periodical |
| Magazine / Source | Vnitřní lékařství |
| MU Faculty or unit | |
| Citation | |
| Field | Oncology and hematology |
| Keywords | autoimmunity; bendamustine; bortezomibe; hyperviscosity; ibrutinib; cryoglobulinemia; monoclonal immunoglobulin related disease; cold agglutinin disease; rituximab; Waldenström macroglobulinemia |
| Description | Waldenström macroglobulinemia is defined by the presence of monoclonal immunoglobulin IgM type (M-IgM) and evidence of lymphoplasmacytic bone marrow infiltration. The disease has an indolent course, the treatment is only initiated when the disease has begun to damage its carrier. The following symptoms are regarded as proven indications for initiating therapy: B symptoms, symptomatic lymphadenopathy, splenomegaly, anemia with hemoglobin below 100 g / l or thrombocytopenia < 100 x 109/l, caused by lymphoplasmacytic bone marrow infiltration. Frequent indications for initiating treatment include clinical evidence of hyperviscosity or cryoglobulinemia. M-IgM tends to have a character of autoantibody reaching up to 50 %, which may harm the organism, and therefore any proven damage to the organism by an autoimmune activity of M-IgM is also an indication for treatment. The text includes an overview of rare and very rare types of damage to the organism by M-IgM autoimmune activity. A combination of rituximab, cyclophosphamide and dexamethasone (RCD) is recommended for the initial treatment, possibly extended to R-CHOP regimen (rituximab, cyclophosphamide, vincristine, doxorubicin and prednisone). In our cohort of 43 patients the therapy involving a combination of R-CHOP achieved 3 (8.1 %) complete remissions and 31 (83.8 %) partial remissions. The remission in 75 % of the patients lasted more than 3 years. |