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Histology of intestinal whipple's disease revisited

A study of 48 patients

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Abstract

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.

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Authors and Affiliations

  1. Institute of Pathology, University of Heidelberg, Im Neuenheimer Feld 220, D-69120, Heidelberg, Germany

    A. von Herbay & H. F. Otto

  2. Institute of Hygiene and Medical Microbiology, University of Heidelberg, Heidelberg, Germany

    M. Maiwald & H. -J. Ditton

Authors
  1. A. von Herbay
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  2. H. F. Otto
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  3. M. Maiwald
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  4. H. -J. Ditton
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Correspondence to A. von Herbay.

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Dedicated to Prof. Dr. med. Gerhard Seifert, Emeritus Director of the Institute of Pathology, University of Hamburg, Germany, on the occasion of his 75th birthday

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von Herbay, A., Otto, H.F., Maiwald, M. et al. Histology of intestinal whipple's disease revisited. Vichows Archiv A Pathol Anat 429, 334–343 (1996). https://doi.org/10.1007/BF00198437

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  • DOI: https://doi.org/10.1007/BF00198437

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