[Whipple's disease. Do we think of it enough?]. / Whipple-kór. Vajon gondolunk-e mindig rá?

ABSTRACT

UNLABELLED Whipple's disease is a chronic, systemic, relapsing bacterial illness, which is always fatal without treatment, and means a diagnostic challenge for both clinicians and pathologists. It occurs in Caucasian, white, middle-aged men in 80 % of the cases. CASE REPORT The authors present three cases, and review the etiology, clinical features, presumed pathomechanism and the possibilities of treatment. CONCLUSION: Several immune-mechanisms have been discussed in the background of Whipple's disease, but their primary or secondary role is yet undetermined. First of all, this is a gastrointestinal disease; however, extraintestinal symptoms may be present many years before the final diagnosis. The histological hallmark for the diagnosis is the presence of numerous macrophages in the duodenal mucosa showing periodic acid-Schiff (PAS)-positive inclusions, and the polymerase chain reaction. The choice of antibiotics and the length of the treatment is empiric, but in most cases there is an immediate response to therapy. Relapses are common, especially the involvements of central nervous system.