Benign hypergammaglobulinemic purpura of Waldenström associated with Sjögren's syndrome. Case report and review of immunologic aspects

The American Journal of Medicine
J E FerreiroE Gould

Abstract

Since its first description, fewer than 150 cases of benign hypergammaglobulinemic purpura of Waldeström have been reported. There is a preponderance of females with this disorder, which is characterized by long-standing purpuric vasculitic lesions usually in the lower extremities, increased sedimentation rate, anemia, leukopenia, and hyperglobulinemia with a normal clotting process. Numerous associations have been shown between this and other systemic disorders such as Sjögren's syndrome, systemic lupus erythematosus, a rheumatoid arthritis. A 40-year-old woman is described who had an 18-year history of recurrent purpura and increased IgG levels. Skin biopsy showed acute vasculitis, and immunofluorescent study revealed fibrinogen precipitation and C3 deposition. Serologic evaluation demonstrated the presence of rheumatoid factor and antinuclear antibodies (1:80). Raji assay showed increased circulating immune complexes, and the T cell subsets were normal. The purpura was associated with symptoms and physical findings of Sjögren's syndrome. On the basis of serologic and skin biopsy findings, an autoimmune origin of the disease is postulated.

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Citations

Dec 6, 2008·Clinical Rheumatology·Elaine C JollyKen Farrington
Sep 1, 2005·Digestive Diseases and Sciences·Jason SlateRichard S Phillips
Dec 11, 1999·Clinics in Dermatology·G HautmannT M Lotti
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Oct 17, 2006·The Journal of Dermatology·Maki Maeda-TanakaSachiko Miyagawa
Mar 14, 2002·American Journal of Hematology·Tawee Tanvetyanon, John C Leighton
Aug 10, 2010·Clinics in Chest Medicine·Maria KokosiKristin B Highland

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