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Waldenström Macroglobulinemia Caused by Extranodal Marginal Zone B-Cell Lymphoma
A Report of Six Cases

Riccardo Valdez MD, William G. Finn MD, Charles W. Ross MD, Timothy P. Singleton MD, Joseph A. Tworek MD, Bertram Schnitzer MD
DOI: http://dx.doi.org/10.1309/6WPX-66CM-KGRH-V4RW 683-690 First published online: 1 November 2001


Waldenström macroglobulinemia (WM) and its associated hyperviscosity syndrome (HVS) are generally caused by lymphoplasmacytoid lymphoma or other small B-cell lymphoproliferative disorders. WM associated with extranodal marginal zone B-cell–mucosa-associated lymphoid tissue lymphoma (EMZL/MALT-type) has not been emphasized. We describe 4 men and 2 women (age, 40–79 years) with clinical and laboratory manifestations of WM and EMZL/MALT-type involving one or more sites: lung, pericardium/pleura, ocular adnexa, nasopharynx, minor salivary gland, glossopharyngeal fold, skin, and stomach. The following immunophenotypic patterns were observed: CD20+, 6; CD43+, 3; kappa light chain restriction, 5; and lambda light chain restriction, 1. All were negative for CD5, CD10, and cyclin D1 expression. A clonal paraproteinemia was present in each (IgM kappa, 4; IgM lambda, 1; biclonal IgM kappa/IgA kappa, 1). All 4 patients tested had elevated plasma viscosity; clinical HVS occurred in 3, and 2 required emergency plasmapheresis. These findings suggest that EMZL/MALT-type can cause WM and that the laboratory evaluation of EMZL/MALT-type should include serum protein electrophoresis/immunofixation, and plasma viscosity measurements and urine immunofixation in select cases. EMZL/MALT-type should be considered in the differential diagnosis in patients with clinicopathologic features of WM.

Key Words:
  • Extranodal marginal zone B-cell lymphoma
  • Mucosa-associated lymphoid tissue lymphoma
  • MALT lymphoma
  • Non-Hodgkin lymphoma
  • Waldenström macroglobulinemia
  • Hyperviscosity syndrome