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    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

  • 1

    Bedu-Addo G, Bates I, 2002. Causes of massive splenomegaly in Ghana. Lancet 360 :449–454.

  • 2

    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.

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    • Export Citation
  • 3

    Ziegler JL, 1973. Cryoglobulinaemia in tropical splenomegaly syndrome. Clin Exp Immunol 15 :65–78.

 

 

 

 

Hyper-reactive Malarial Splenomegaly

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  • 1 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

Figure 1.
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

Citation: The American Journal of Tropical Medicine and Hygiene Am J Trop Med Hyg 78, 2; 10.4269/ajtmh.2008.78.186

*

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@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@itsa.ucsf.edu.

REFERENCES

  • 1

    Bedu-Addo G, Bates I, 2002. Causes of massive splenomegaly in Ghana. Lancet 360 :449–454.

  • 2

    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.

    • Search Google Scholar
    • Export Citation
  • 3

    Ziegler JL, 1973. Cryoglobulinaemia in tropical splenomegaly syndrome. Clin Exp Immunol 15 :65–78.

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