Cause:Tropheryma whippelii,Whipple's bacillus
Pathophys:Infection of small intestinal mucosal layer causes lymphatic blockage, which in turn causes fatty acid malabsorption, probably at lamina propria level; infection also affects other body systems, eg, CNS, etc
Sx:Diarrhea, steatorrhea, and malabsorption; postprandial pain; polyserositis including arthritis in 70%, frequently the presenting sx; fever, weight loss; lymphadenopathy; hyperpigmentation of skin; blurred vision from uveitis, may be only sx (Nejm 1995;332:363)
Si:As above
Myocarditis and aortic insufficiency (Nejm 1981;305:995), culture negative endocarditis; CNS, including gaze paresis, nystagmus, myoclonus, polydipsia, hypersomnolence, all of which may be a continuing problem if primary sx are treated with an antibiotic that doesn't cross blood-brain barrier (Nejm 1979;300:907)
Lab:
Bact:Gram-pos bacillus of actinomycetes group; identifiable by PCR; and culturable now (Nejm 2000;342:620)
Hem:CBC peripheral smear may show red stippled rbc's (organisms in the red cells) (Nejm 1994;331:1343)
Path:Intestinal bx shows diagnostic changes of enlarged villi; foamy, carbohydrate-filled macrophages staining positive with PAS; and fat in mucosa, lacteals, and lymph nodes (Nejm 1971;285:1470); r/o AIDS or M. aviumintestinal infection; and, if has noncaseating granulomata, sarcoid
Rx:(Nejm 1996;335:26) Procaine penicillin 1.2 million U im qd × 14 d + streptomycin 1 gm im qd × 14 d + Tm/S bid prophylaxis continuously