Waldenstrom s macroglobulinemia

This chapter reviews our current understanding of the mechanism of action of monoclonal antibody (especially rituximab), as well as the role of Fey receptor and Fey receptor gene polymorphisms, and their impact on treatment outcomes in hematologic malignancies including follicular lymphoma (FL), diffuse large B-cell lymphoma (DL-BCL), Waldenstrom s macroglobulinemia (WM), and chronic lymphocytic leukemia (CLL). 

Waldenstrom s macroglobulinemia (WM) is an uncommon lymphoid malignancy characterized by IgM monoclonal gammopathy and intertrabecular marrow infiltration by small lymphocytes expressing CD20 antigen. Currently, rituximab can be used for the treatment of WM with response rates between 20% and 50% (62,63). 

Johnson SA. Advances in the treatment of Waldenstrom s macroglobulinemia. Expert Rev Anticancer Ther 2006 6 329-334. 

Treon SP, Hansen M, Branagan AR et al. Polymorphisms in FcgammaRIIIA (CD16) receptor expression are associated with clinical response to rituximab in Waldenstrom s macroglobulinemia. J Clin Onco/2005 23 474 81. 

Krumdieck R, Shaw DR, Huang ST, Poon MC, Rustagi PK. Hemorrhagic disorder due to an isoniazid-associated acquired factor XIII inhibitor in a patient with Waldenstrom s macroglobulinemia. Am J Med 1991 90(5) 639 5. 

Intensive plasma exchange has been studied in the treatment of numerous diseases, above all in Waldenstrom s macroglobulinemia, hypercholesterolemia, hyperviscosity syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, myasthenia... 

Mitsiades, C.S., N. Mitsiades, P.G. Richardson, S.P. Treon and K.C. Anderson. Novel biologically based therapies for Waldenstrom s macroglobulinemia. Semin. Oncol. 30 309-312, 2003. 

Malignant immunocytomata 68% IgG—myelomatosis 28% IgA—myelomatosis 18% Bence Jones only—myelomatosis 11% IgD— myelomatosis 14% IgM—myelomatosis 17% No paraprotein myelomatosis 5% Waldenstrom s macroglobulinemia... 

In summary Waldenstrom s macroglobulinemia seems to be a slow-growing infiltrating, widespread lymphoma whose protein production is thereby allowed to gain viscosity syndrome levels before the tumor does any direct harm. With adequate control, the prognosis is good, up to 10 years. 

M2. MacKenzie, M. R., Brown, E., Fudenberg, H. H., and Goodenday, L., Waldenstrom s macroglobulinemia correlation between expanded plasma volume and increased sei-um viscosity. Blood 36, 394-408 (1970). 

The ISO-DALT technique has been used to investigate the pattern of proteins in sera from patients with, for example, Hodgkin s disease, Waldenstrbm s macroglobulinemia, and myeloma (L4, T3). In addition, it has proved a valuable tool in the study of the protein matrix of animal-and human-based quality control sera (C7). The technique has also been successfully applied to the analysis of particular proteins, e.g., the apoli-poproteins (Zl). Complementary studies, using nondenatured samples of sera from patients with, for example, myeloma, WaldenstrOm s macroglobulinemia, hepatic cirrhosis and of the lipoproteins have also been carried out (E3, E4, L3) and are reviewed by Latner (L2). 

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