Whipple’s disease is a chronic inflammatory systemic disorder in which all organs can be invaded by the rod-shaped bacterium Tropheryma whipplei. It is a rare disease and frequently misdiagnosed, though there is no valid estimate of its actual incidence and prevalence. Only about 1,000–1,500 cases have been reported. The clinical course of untreated Whipple’s disease can include three stages: (1) a non-specific prodromal stage which includes migratory polyarthralgias; (2) a classic abdominal manifestation which involves weight loss, weakness, chronic diarrhea, and abdominal pain; and (3) a generalized stage characterized by steatorhea, cachexia, lymphadenopathy, hyper-pigmentation, and cardiovascular, pulmonary, and neurological dysfunction. The authors describe a case of a 39-year-old male patient with about a year’s history of generalized adenopathy, inappetence, weight loss, progressive weakness, subfebrilities, and convulsive abdominal pain. Following primary exclusion of a tumor disease, a lymph node biopsy demonstrated a typical picture of a granulomatous inflammation—Whipple’s lymphadenitis with partial exemption of the Gram reaction, and stain features corresponding to T. whipplei, which is regarded as the etiological agent causing this disorder. Further tests confirmed the generalized form of the disorder, affecting the lymphatic tissues, gastrointestinal system, respiratory system, and nervous system, with sensory and motor polyneuropathy. HLA-B27 antigen, which is frequent among those with Whipple’s disease, was also present. Following treatment for three months with antibiotics a significant reduction of the changes typical of Whipple’s disease was found upon follow-up biopsy, hence we assume the applied therapy was successful. In our case study we emphasize the atypical course of the disease with dominant generalized lymphadenopathy and only mild gastrointestinal symptoms.
This is a preview of subscription content, log in to check access.
Whipple GH (1907) A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues. Bull Johns Hopkins Hosp 18:382–391Google Scholar
Cooper GS, Blades EW, Remler BF, Salata RA, Bennert KW, Jacobs GH (1994) Central nervous system Whipple’s disease: relapse during therapy with trimethoprim-sulfamethoxazole and remission with cefixime. Gastroenterology 106:782–786PubMedGoogle Scholar
Black-Schaffer B (1949) The tinctorial demonstration of a glycoprotein in Whipple’s disease. Proc Soc Exp Biol Med 72:225–227PubMedGoogle Scholar
Paulley JW (1952) A case report of Whipple’s disease (intestinal lipodystrophy). Gastroenterology 22:128–32PubMedGoogle Scholar
La Scola B, Fenollar F, Fournier PE, Altwegg M, Mallet MM, Raoult D (2001) Description of Tropheryma whipplei gen. nov., sp. nov., the Whipple’s disease bacillus. Int J Syst Evol Microbiol 51:1471–1479PubMedGoogle Scholar
Vital-Durand D, Lecomte C, Cathebras P, Rousset H, Godeau P (1997) Whipple disease clinical review of 52 cases. The SNFMI research group on Whipple’s disease. Societe Nationale Francaise de Medecine Interne. Medicine (Baltimore) 76:170–184. doi:10.1097/00005792-199705000-00003CrossRefGoogle Scholar
Dobbins WO. Whipple’s disease. Charles C Thomas, Springfield 1987, IIIGoogle Scholar
Gunia S, Behrens MH, Stosiek P (2004) A rare course of Whipple’s disease with atypical cardiac manifestation. AMPIS 112:222–224Google Scholar
Maiwald M, Ditton HJ, von Herbay A et al (1996) Reassessment of the phylogenetic position of the bacterium associated with Whipple’s disease and determination of the 16S-23S ribosomal intergenic spacer sequence. Int J Syst Bacteriol 46:3292–3297CrossRefGoogle Scholar
Di Stefano M, Jorizzo RA, Brusco G, Ceccheti L, Sciarra G, Loperfido S, Brandi G, Gasbarrini G, Corazza GR (1998) Bone mass and metabolism in Whipple’s disease: the role of hypogonadism. Scand J Gastroenterol 33:247–252. doi:10.1080/00365529850172548CrossRefGoogle Scholar
Keinath RD, Merell DE, Vlietstra R, Dobins WO (1985) III. Antibiotic treatment and relapse in Whipple’s disease: long-term follow-up of 88 patients. Gastroenterology 88:1967–1973Google Scholar