Histology of intestinal Whipple's disease revisited. A study of 48 patients

Virchows Arch. 1996 Dec;429(6):335-43. doi: 10.1007/BF00198437.


Whipple's disease is an infectious disorder with intestinal and extra-intestinal manifestations. We reinvestigated the intestinal histology in a series of 48 patients (10 females, 38 males; mean age 56.5 years, standard deviation of the mean +/- 11.2 years). A total of 126 biopsy samples, obtained prior to, during, and after therapy, were evaluated by light microscopy. In 43 patients (90%), histology was consistent with common descriptions, while it was uncommon in 3 patients (6%), and non-diagnostic in 2 patients (4%). During treatment, several alterations occurred. Apart from a continuous decrease in PAS-positive macrophages, the pattern of mucosal infiltration changed from diffuse to patchy. Moreover, the cytological aspects of PAS-positive macrophages changed substantially, and this change was used to propose four different subtypes. Initially, subtype 1 macrophages predominated (74%), but showed a gradual decrease within a few months of therapy. After 15 months, subtype 3 and subtype 4 macrophages predominated (< 80%). In 7 of 9 patients followed over long periods some subtype 3 or subtype 4 macrophages persisted. It is concluded that at diagnosis and during treatment the intestinal histology of Whipple's disease is heterogeneous. A few PAS-positive macrophages commonly persist at long-term follow-up. This and other features suggest the presence of a persistent immune defect.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Anti-Bacterial Agents / therapeutic use
  • Follow-Up Studies
  • Giardia / isolation & purification
  • Humans
  • Intestinal Diseases / drug therapy
  • Intestinal Diseases / parasitology
  • Intestinal Diseases / pathology*
  • Intestinal Mucosa / parasitology
  • Intestinal Mucosa / pathology
  • Macrophages / pathology
  • Periodic Acid-Schiff Reaction
  • Remission Induction
  • Whipple Disease / drug therapy
  • Whipple Disease / parasitology
  • Whipple Disease / pathology*


  • Anti-Bacterial Agents