Cutaneous macroglobulinosis with monotypic plasma cells: A specific manifestation of Waldenstr?m macroglobulinemia

摘要

Waldenstrom macroglobulinemia occurs in a subset of patients with lymphoplasmacytic lymphoma, which is a chronic, indolent tumor of mature B-cells, plasmacytoid cells and plasma cells infiltrating the bone marrow and sometimes lymph nodes and spleen. In addition to bone marrow involvement, the diagnosis of Waldenstrom macroglobulinemia requires the presence of an immunoglobulin M (IgM) monoclonal gammopathy of any concentration. Signs and symptoms of the entity are attributable to tumor infiltration of the bone marrow (resulting in cytopenias, including anemia); elevated circulating IgM (resulting in increased protein concentration with vascular resistance and hyperviscosity, interference with coagulation and yielding a bleeding diathesis, and precipitation at cold temperatures resulting in cryoglobulinemia); tissue deposition of IgM (including deposits in glomerular loops, intestine, and skin); and autoantibody activation by IgM (resulting in peripheral neuropathy, autoimmune hemolytic anemia, and Schnitzler syndrome).