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Waldenström's Macroglobulinemia

Gerburg Wulf, M.D., and Stephen Pool, M.D.

N Engl J Med 2001; 344:816March 15, 2001

Article

Figure 1 A 65-year-old man had an 18-month history of slowly progressive swelling and discoloration of both ears, fatigue, night sweats, weight loss, occipital headaches, and photophobia. On examination, his ears were swollen and purplish and had several small cysts that were neither warm nor tender. The skin of his nose, cheeks, upper lip, and chin was also thickened and discolored (Panel A). He had diffuse peripheral lymphadenopathy. Funduscopy demonstrated tortuous arterioles, distended prominent veins bilaterally with diminished pulsations, and retinal hemorrhages. Serum immunoelectrophoresis revealed a monoclonal IgM spike with an IgM level of 7.7 g per liter and a relative viscosity of 18.8. The patient underwent plasmapheresis. Biopsy of the left ear revealed diffuse dermal lymphoplasmacytic infiltrates (Panel B; hematoxylin and eosin, ×100), with numerous plasma cells showing intranuclear inclusions of immunoglobulin, or Dutcher bodies (arrows in Panel C; hematoxylin and eosin, ×500). Examination of a bone marrow aspirate and a bone marrow–biopsy specimen showed dense infiltration with lymphoplasmacytic cells expressing monotypic IgM lambda light chains, thus confirming the diagnosis of Waldenström's macroglobulinemia. The patient's symptoms improved dramatically with plasmapheresis and the institution of chemotherapy with fludarabine, and the swelling of his ears resolved.

Gerburg Wulf, M.D.
Stephen Pool, M.D.
Beth Israel Deaconess Medical Center, Boston, MA 02215

Citing Articles (1)

Citing Articles

  1. 1

    Jonathan W. Said. (2002) Nodal Lymphoplasmacytic Lymphoma/Immunocytoma: An Unusual Form of Non-Hodgkin Lymphoma With Specific Pathologic and Immunohistochemical Features, With Particular Reference to Pitfalls in its Differential Diagnosis. Pathology Case Reviews 7:3, 83-87
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