Vnitr Lek 2008, 54(1):68-83

The treatment of Waldenström macroglobulinemia and the therapy of diseases caused by monoclonal IgM gammaglobulin

Z. Adam1,*, V. Ščudla2, M. Krejčí1, Z. Kořístek1
1 Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc.
2 III. interní klinika Lékařské fakulty UP a FN Olomouc, přednosta prof. MUDr. Vlastimil Ščudla, CSc.

Waldenström macroglobulinemia is defined by the presence of IgM type monoclonal immunoglobulin and histological prove of lymphoplasmocytary lymphoma in the bone marrow. Alkylating cytostatic drugs, chlorambucil or cyclofosfamide in monotherapy have been typically used for its treatment. Similarly to other lymphoproliferative diseases with a low degree of aggressiveness, purine analogues (fludarabine and 2-chlordeoxyadenosine) have a faster treatment response and a higher number of treatment responses, especially if administered in combination with alkylating drugs. The monoclonal antibody rituximab (R) also brings considerable improvement to patients suffering from the disease. It is good for cytopenic patients on monotherapy, and for non-cytopenic patients, in combination with other drugs, both with R-CHOP and with a purine analogue and an alkylating cytostatic drug. In recent years, new drugs for the treatment of this disease have been tested (bortezomib, thalidomid, revlimid and others). High-dose chemotherapy with autologous transplantation has a higher treatment response rate than classical chemotherapy. It is recommended in case of insufficient effect of conventional treatment or early relaps. Young patients with suitable profile and insufficient response to conventional treatment can also qualify for allogenic transplantation; graft response against the disease has been observed in almost all patients who underwent allogenic transplantation. However, treatment is often necessary also in diseases caused by IgM monoclonal immunoglobulin, irrespective of it being formed by a malignant or a benign clone, because the characteristics of IgM monoclonal immunoglobulin may endanger the patient's integrity. The article provides an overview of treatment options for Waldenström macroglobulinemia and diseases caused by monoclonal IgM.

Keywords: Waldenström macroglobulinemia; diseases caused by monoclonal immunoglobulin; disease of cold agglutinins; cryoglobulinemia; hyperviscosity

Received: July 18, 2007; Accepted: September 26, 2007; Published: January 1, 2008  Show citation

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Adam Z, Ščudla V, Krejčí M, Kořístek Z. The treatment of Waldenström macroglobulinemia and the therapy of diseases caused by monoclonal IgM gammaglobulin. Vnitr Lek. 2008;54(1):68-83.
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