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  • 1 Department of Medicine, Hospital of Zala County, Zalaegerszeg, Hungary
  • 2 Department of Pathology, Hospital of Zala County, Zalaegerszeg, Hungary
  • 3 Zrínyi M. str. 1, H-8900, Zalaegerszeg, Hungary
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Abstract

Whipple's disease is a chronic, systemic, relapsing bacterial illness, which always has a fatal outcome without treatment. It represents a significant diagnostic challenge for both clinicians and pathologists. In 80% of cases, the disease affects middle-aged white men belonging to the Caucasian race. Case report: The authors present three cases and review the aetiology, clinical features, presumed pathomechanism and the possibilities of treatment. Conclusion: Several immunologic mechanisms underlying Whipple's disease have emerged, but their primary or secondary nature has not yet been elucidated. First of all, this is a gastrointestinal disease; however, extraintestinal symptoms may often precede final diagnosis by several years. The histological hallmark for the diagnosis is the presence of numerous macrophages in the duodenal mucosa showing periodic acid — Schiff (PAS)-positive inclusions as well as polymerase chain reaction. The choice of antibiotics and the duration of therapy are empirical, but in most cases, there is an immediate response to the treatment. Relapses are common, particularly in patients with central nervous system involvement.

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