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Whipple's disease

A review of the literature and report of fifteen patients

Whipplesche Krankheit

Literaturübersicht und Mitteilung von 15 Fällen

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Summary

15 necropsied cases ofWhipple's disease are presented and compared with the literature. Most clinical manifestations of the disease are the result of three basic tissue alterations: a) Deposition of a strongly PAS-positive substance in macrophages of the small intestines, regional lymph nodes, and, to a lesser degree, extramesenteric visceral and peripheral lymph nodes, valvular endocardium, spleen, and other organs. Histochemical studies of the deposited material suggest a complex mucopolysaccharide with a protein and lipid component. b) Accumulation of a lymph-like lipid-containing substance within lymphatics and in extralymphatic cystic spaces of the intestinal mucosa and regional lymph nodes. c) Fibrosis in the regional lymph nodes, spleen, and mesenteric lymphatics as late phenomenon, probably incited by the deposited material. The degree of impairment of lymph drainage is clinically reflected in the severity of abdominal symptoms. Early diagnosis is best accomplished by transoral small bowel biopsy or by the examination of peripheral lymph nodes.

Zusammenfassung

Die makroskopischen und histologischen Untersuchungsbefunde von 15 Fällen von Whipplescher Krankheit werden mitgeteilt und mit den Literaturbefunden verglichen. Die klinischen Erscheinungen lassen sich auf drei Grundprozesse zurückführen: 1. Speicherung einer stark PAS-positiven Substanz in den Makrophagen des Dünndarms, den regionalen Lymphknoten und, in geringerem Grade, in den übrigen visceralen und peripheren Lymphknoten, im Endokard, in der Milz und anderen Organen; histochemische Untersuchungen machen es wahrscheinlich, daß das gespeicherte Material ein komplexes Mucopolysaccharid darstellt mit einer Eiweiß- und einer Lipoidkomponente. 2. Anreicherung einer lymphähnlichen, Lipoide enthaltenden Substanz in den Lymphbahnen und extralymphatischen Spalträumen der Dünndarmschleimhaut und der regionalen Lymphknoten. 3. Fibrose der regionalen Lymphknoten, der Milz und mesenterialen Lymphbahnen, wahrscheinlich ausgelöst durch die Speicherung der Mucopolysaccharide; die dadurch verursachte Störung des Lymphabflusses spiegelt sich in der Schwere der Bauchsymptome wider. Die Frühdiagnose kann am zuverlässigsten durch die histologische Untersuchung einer transoralen Dünndarmbiopsie oder eines peripheren Lymphknotens gestellt werden.

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Authors and Affiliations

  1. Department of Pathology, Armed Forces Institute of Pathology, USA

    Franz M. Enzinger & Elson B. Helwig

  2. Veterans Administration Central Laboratory for Anatomic Pathology and Research, Washington, D.C.

    Franz M. Enzinger & Elson B. Helwig

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  1. Franz M. Enzinger
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  2. Elson B. Helwig
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Assistant Chief, Endocrine and Soft Tissue Branch, AFIP.

Chief, Department of Pathology, and Branch of Gastrointestinal Pathology, AFIP.

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Enzinger, F.M., Helwig, E.B. Whipple's disease. Virchows Arch. path Anat. 336, 238–269 (1963). https://doi.org/10.1007/BF00957912

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