Whipple's Disease

In 1907 Whipple made an autopsy doctor, who died from an unknown, chronic illness. The patient was suffering from exhaustion, diarrhea, dry cough and migratory polyarthritis. In the study of the abdominal cavity Whipple have discovered an unusual deposition of fatty substances in the wall of the small intestine and mesenteric nodes. When microscopy were found special "frothy" cells close to the macrophages. Whipple called the disease "intestinal lipodystrophy, and later it became known by the name of the author, her first described. Currently available in the literature for over 100 observations Whipple's disease.
In the modern view, Whipple's disease is a systemic disease that primarily affects the small intestine, the lymphatic system and synovial joints cover. Thus there is infiltration of tissues "frothy" macrophages containing glycoproteins, and cystic enlargement of lymph vessels containing lipids (Haubrich and others, 1964; Sheehy, Floch, 1964).
Whipple's disease belongs to a rare nosologic forms.
Men in 8 times more often than women are affected by this disease. Mainly affects people of middle age, and persons older than 50 years.
The etiology and pathogenesis. The etiology of the disease remains unknown. Many researchers attribute the Whipple's disease to the group kollagenozov; indeed the main pathogenetic link of the pathological process are violations in the sphere of exchange of mucopolysaccharides. With a second change occurs in the epithelial cells of the small intestine, which lead to the occurrence of the syndrome malabsorbtion". The dissemination process into going through the lymph system, which provides a generalization of the process and the defeat of the serous membranes.
Recently Trier et al. (1965), has received new information concerning the etiology of the disease Whipple. By studying the results of aspiration biopsy of the small intestine, they found in its own layer of mucous membrane of bacteria with the capsule, which most clearly been able to study only in electron microscopy. These bacteria were presented within macrophages and neutrophils. The authors point to the specific character of microbes in this disease. During periods of exacerbation of the number of bacteria in the intestinal wall clearly increased. Sherris et al. (1965) tried to allocate microbes in culture and to vaccinate their animals, but all their attempts were unsuccessful. Perhaps these germs and cause proliferation reticular cells in the intestinal wall.
Pathological anatomy. If Whipple's disease is defined quite characteristic morphological picture of destruction of the small intestine.
Intestinal wall is compacted and stretched, mucous membrane has grained surface, thickened fibers and shortened. Are determined the increased mesenteric lymph nodes thickened and mesentery.
Histological examination of the greatest diagnostic value is detected in its own layer of the small intestine specific macrophages with foam protoplasm and special inclusions (Taurus Sieracki) in the form of grains (Fig. 34). In 1949 Black-Schaffer found that macrophages intensively painted reagent Schiff (PAS-reaction). Detection of such cells is considered pathognomonic for Whipple's disease. In recent years, using electron microscopy were found encapsulated bacillophobia bullock under basal membrane of epithelial cells of the small intestine (Fig. 35).

Fig. 34. Microphoto jejunal mucosa with the Whipple's disease (HC. 250).
Visible multiple macrophages in its own layer intestinal villi.
Fig. 35. Microphoto intestinal villi at the Whipple disease under electron microscope (HC. 10 000).
Visible bacillophobia Taurus in its own layer under the basal membrane. Private shell is left, epithelial cell on the right.

PAS-positive macrophages are found not only in the small intestine, but in the serous membranes, lymph nodes, synovial lining of the joints. In addition, in the lymph nodes and the mesentery formed granulomas that have cavities filled with fat lipogenesis.