Am. J. Trop. Med. Hyg., 78(2), 2008, pp. 186-187
Copyright © 2008 by The American Society of Tropical Medicine and Hygiene
Hyper-reactive Malarial Splenomegaly
John L. Ziegler*
University of California—San Francisco, San Francisco, California
Hyper-reactive malarial splenomegaly (HMS) is a syndrome of massive, unexplained splenomegaly occurring in a malarious region, accompanied by lassitude, fever, weight loss, hypergammaglobulinemia (especially IgM), and cryoglobulinemia. A clinical response to prolonged antimalarial prophylaxis is diagnostic,1 but the pathogenesis is unclear. In some patients, the condition will progress to splenic lymphoma with villous lymphocytes.2 Figure 1A
shows a 14-year-old Ugandan girl with HMS with the spleen profile outlined by traditional therapeutic scarifications. Figure 1B demonstrates cryoglobulinemia. The left vial contains HMS plasma kept overnight at 25°C, and the right vial shows a cloudy cryoprecipitate (containing IgM, IgG, complement, and rheumatoid factor) in the same plasma kept overnight at 4°C. Figure 1C shows typical hepatic sinusoidal lymphocytosis in a liver biopsy from an HMS patient, and Figure 1D displays IgM lining the hepatic sinusoids revealed by fluorescein-tagged goat anti-IgM antibody.3
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FIGURE 1. Hyper-reactive malarial splenomegaly (HMS), (A) showing splenic scarring; (B) cryoglobulinemia; (C) hepatic sinusoidal lymphocytosis in a patient with HMS; and (D) IgM, revealed by fluorescein-tagging. This figure appears in color at www.ajtmh.org
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Received December 10, 2007.
Accepted for publication December 17, 2007.
* Address correspondence to John L. Ziegler, Professor of Medicine, Emeritus, University of California–San Francisco, 2340 Sutter Street, N424, San Francisco, CA 94143-0808. E-mail: Ziegler{at}itsa.ucsf.edu 
Authors’ address: John L. Ziegler, Professor of Medicine, Emeritus, University of California—San Francisco, 2340 Sutter Street, N424, San Francisco, CA 94143-0808, Telephone: +1 (415) 502-1883, Fax: +1 (415) 476-8218, E-mail: Ziegler{at}itsa.ucsf.edu.
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REFERENCES
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- Bedu-Addo G, Bates I, 2002. Causes of massive splenomegaly in Ghana. Lancet 360: 449–454.[ISI][Medline]
- Bates I, Bedu-Addo G, Rutherford TR, Bevan DH, 1997. Circulating villous lymphocytes—a link between hyperreactive malarial splenomegaly and splenic lymphoma. Trans R Soc Trop Med Hyg 91: 171–174.[ISI][Medline]
- Ziegler JL, 1973. Cryoglobulinaemia in tropical splenomegaly syndrome. Clin Exp Immunol 15: 65–78.[ISI][Medline]
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