Poškození způsobená depozity monoklonálního imunoglobulinu typu IgM a volnými lehkými řetězci u Waldenströmovy makroglobulinémie. Popis případu a přehled literatury

Abstract

Summary

Disorders induced by deposits of monoclonal immunoglobulin IgM and Free Light Chain in Waldenström’s macroglobulinaemia. Cases report and review of literature.

Waldenström macroglobulinaemia (WM) is a low grade B-cell lymphoproliferative disorder characterised by an immunoglobulin IgM monoclonal gammopathy and bone marrow infiltration by lymphoplasmacytic lymphoma. Clinical features may be related to overall disease burden, such as anaemia, thrombocytopenia, constitutional inflammatory symptoms, or may be directly attributable to the IgM paraprotein. The concentration of monoclonal IgM can vary widely in WM. There is no direct relationship between concentration of monoclonal immunoglobulin IgM and bone marrow infiltration.  The spectrum of monoclonal immunoglobulin IgM related disorders is large because of the diversity of involved organs and pathogenic mechanisms. Lesions commonly result from deposition of all or part of the M-IgM as aggregates, amorphous, crystalline, microtubular, or fibrillar forms. Other mechanisms include autoantibody activity against a tissue antigen, formation of immune complexes, and complement activation. In addition, even a small B-cell clone may absorb biologically active molecules or induce cytokine secretion. In our case report we describe female patient with monoclonal IgM and lambda liver deposition and we discuse frequency and variable forms of disorder caused by deposition of monoclonal IgM and free light chain

Key words: Waldenström´s macroglobulinaemia, hepatomegaly, Monoclonal Immunoglobulin IgM Deposition Disease, macroglobulinosis, amyloidosis.