Summary
Twenty-two patients with Whipple's disease are reviewed (21 male, 1 female; mean age 49.3 years). All but one were diagnosed by small intestinal biopsy. The most frequent clinical symptoms at diagnosis were weight loss (14/21 patients), diarrhea (13/21), arthralgias (13/21), cramping abdominal pain (11/21), and skin pigmentation (8/21), which anteceded the diagnosis by from 6 months to 10 years (median 3.5 years). All but three patients were treated successfully with tetracycline given for at least 2 years. Follow-up was done for a median of 7.7 years (range 1–15 years). Two of 17 patients who were followed for 2 years relapsed, as did 2/11 followed for at least 5 years. One patient relapsed twice. Each relapse could be treated with success. In no case did relapse occur in the central nervous system. Thus, tetracycline appears to be effective in the treatment of Whipple's disease if given for at least 2 years. In two further patients with confirmed central nervous system involvement at diagnosis, who were treated with ampicillin plus chloramphenicol, characteristic SPC cells disappeared from the cerebrospinal fluid. In addition, the recently recognized problems in the differential diagnosis of Whipple's disease and atypical mycobacterial infection in AIDS patients are discussed.
Similar content being viewed by others
Abbreviations
- AIDS:
-
Acquired immune deficiency syndrome
- CNS:
-
Central nrvous system
- PAS:
-
Periodic acid-Schiff's reagent
- SPC:
-
Sickle-form particles containing (cells)
References
Autran B, Gorin I, Leibowitch M, Laroche L, Escande JP, Hewitt J, Marche C (1983) AIDS in a Haitian woman with cardiac Kaposi's sarcoma and Whipple's disease. Lancet I:767–768
Bayless TM (1970) Whipple's disease: newer concepts on therapy. Adv Intern Med 16:171–189
Bayless TM, Knox DL (1979) Whipple's disease: a multisystem infection. N Engl J Med 300:920–921
Black-Schaffer B (1949) The tinctorial demonstration of a glycoprotein in Whipple's disease. Proc Soc Exp Biol Med 72:225–227
Bolt RJ, Pollard HM, Standaert L (1958) Transoral small bowel biopsy as an aid in the diagnosis of malabsorption states. N Engl J Med 259:32–34
Buchholz K, Maintz J, Otto HF (1974) Klinisch-immunologische und elektronenmikroskopische Untersuchungen bei Morbus Whipple. Klin Wochenschr 52:672–677
Comer GM, Brandt LJ, Abissi CJ (1983) Whipple's disease: a review. Am J Gastroenterol 78:107–114
Davis DT Jr, McBee JW, Borland JL Jr, Kurtz SM, Ruffin JM (1963) The effect of antibiotic and steroid therapy in Whipple's disease. Gastroenterology 44:112–116
Dobbins WO III (1981) Is there an immune deficit in Whipple's disease? Dig Dis Sci 26:247–252
Dobbins WO III, Kawanishi H (1981) Bacillary characteristics in Whipple's disease: an electron microscopic study. Gastroenterology 80:1468–1475
Enzinger FM, Helwig EB (1963) Whipple's disease: a review of the literature and report of fifteen patients. Virchows Arch Path Anat 336:238–269
Evans DJ, Ali MH (1985) Immunocytochemistry in the diagnosis of Whipple's disease. J Clin Pathol 38:372–374
Feldmann M (1986) Southern Internal Medicine Conference: Whipple's disease. Am J Med Sci 291:56–67
Feurle GE (1983) Morbus Whipple. In: Caspary WE (ed) Handbuch der Inneren Medizin Bd. III/3B Dünndarm. Springer, Berlin Heidelberg New York, pp 85–105
Feurle GE, Utz G, Kies D, Aumüller G (1976) Neurologische Manifestationen des Morbus Whipple. Schweiz Med Wochenschr 106:1642–1646
Francke A, Piesbergen H (1981) Die Therapie des Morbus Whipple. Dtsch Med Wochenschr 106:407–410
Gaertner U, Petzold D, Heisig N, Vogel P, Otto HF (1978) Endoskopische Befunde bei Morbus Whipple. Dtsch Med Wochenschr 103:1740–1742
Gillin JS, Urmacher C, West R, Shike M (1983) DisseminatedMycobacterium avium-intracellulare infection in acquired immunodeficiency syndrome mimicking Whipple's disease. Gastroenterology 85:1187–1191
von Herbay A, Windler F, Heckmayr M, Langkowski R, Kraas E, Otto HF (1987) Abdominaler Pseudotumor als klinische Manifestation eines Morbus Whipple. T-Zell-Index als Indikator der Krankheitsaktivität und Parameter der Therapiedauer? Dtsch Med Wochenschr 112:1621–1625
Hofmann W, Nassal J, Dalchow W, Schimpf K, Reiss B, Möller P (1988) Generalized mycobacterial histiocytosis induced byMycobacterium intracellulare serotype 8 (syn. DAVIS) mimicking Hodgkin's disease in a young man with AIDS. Virchows Arch Path Anat (in press)
Keinath RD, Merrell DE, Vlietstra R, Dobbins WO III (1985) Antibiotic treatment and relapse in Whipple's disease. Gastroenterology 88:1867–1873
Knox DL, Bayless TM, Pittman FE (1976) Neurologic disease in patients with treated Whipple's disease. Medicine 55:467–476
Kölbel HK (1984) Electron microscopy (of mycobacteria). In: Kubica GP, Wayne LG (eds) The mycobacteria, a sourcebook, part A. Dekker, New York Basel, pp 249–300
Maizel H, Ruffin JM, Dobbins WO III (1970) Whipple's disease: a review of 19 patients from one hospital and a review of the literature since 1950. Medicine 49:175–205
Otto HF, Begemann F (1970) Vergleichende ultrastrukturelle und klinische Studie zum Ablauf des M. Whipple. Virchows Arch Abt A Path Anat 350:368–388
Otto HF, Caselitz J (1982) Morbus Whipple — eine systemische Infektionskrankheit? Dtsch Med Wochenschr 107:123–124
Otto HF, Siemssen S, Sill V (1972) Zur Differentialdiagnose von Morbus Whipple und Sarkoidose. Dtsch Med Wochenschr 97:1343–1347
Paulley JW (1952) A case of Whipple's disease (intestinal lipodystrophy). Gastroenterology 22:128–133
Roth RI, Owen RL, Keren DF (1983) AIDS withMycobacterium avium-intracellulare lesions resembling those of Whipple's disease (letter). N Engl J Med 309:1324–1325
Ryser RJ, Locksley RM, Eng SC, Dobbins WO III, Schoenknecht FD, Rubin CE (1984) Reversal of dementia associated with Whipple's disease by trimethoprim-sulfamethoxazole, drugs that penetrate the blood-brain barrier. Gastroenterology 86:745–752
Sieracki JC (1958) Whipple's disease: observations on systemic involvement. I. Cytologic observations. Arch Pathol 66:464–467
Stock KP, Burmester GR, Kalden JR, Schmidt H, Riemann JF (1985) Immunologische und elektronenmikroskopische Befunde eines ungewöhnlichen Falles von M. Whipple. Klin Wochenschr 63:176–183
Strom RL, Gruninger RP (1983) AIDS withMycobacterium avium-intracellulare lesions resembling those of Whipple's disease (letter). N Engl J Med 309:1323–1324
Wang HH, Tollerud D, Danar D, Hanff P, Gottesdiener K, Rosen S (1986) Another Whipple-like disease in AIDS? N Engl J Med 314:1577–1578
Whipple GH (1907) A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues. Johns Hopkins Hosp Bull 18:382–391
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
von Herbay, A., Otto, H.F. Whipple's disease: A report of 22 patients. Klin Wochenschr 66, 533–539 (1988). https://doi.org/10.1007/BF01736522
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01736522