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Whipple's disease. Demonstration of a persisting monocyte and macrophage dysfunction
R Bjerknes1, S Odegaard, R Bjerkvig
1Gade Institute, Dept. of Pathology, Bergen, Norway.
Abstract:
A patient with Whipple's disease has been followed up for 4 years. Primary involvement was limited to the small intestines, and accumulation of periodic acid-Schiff-positive material, containing typical more or less intact bacillary bodies, was demonstrated within macrophages of affected tissue. After initial oxytetracycline treatment and clinical remission, the patient relapsed, with multiorgan affections. The antibiotic regimen was changed to chloramphenicol, followed by continuous trimethoprim-sulfamethoxazole. Flow cytometric studies showed persisting impairment of monocyte and macrophage intracellular degradation of bacteria during all the 4 years tested. After relapse, reduced activity of several brush border enzymes was demonstrated in distal duodenal biopsy specimens. After 17 months of continuous trimethoprim-sulfamethoxazole therapy complete clinical remission, regression of histopathologic abnormalities, and restoration of duodenal enzyme activities had occurred. The results demonstrate a persisting dysfunction of mononuclear phagocytes from a patient with Whipple's disease, suggesting a primary abnormality of cell-mediated immunity which may promote the susceptibility to the causative bacillus.
Insights
This study followed a Whipple's disease patient for 4 years, revealing persistent macrophage dysfunction. Long-term trimethoprim-sulfamethoxazole therapy led to remission, suggesting an underlying immune defect.
Area of Science:
- Gastroenterology
- Immunology
- Infectious Diseases
Background:
- Whipple's disease is a rare bacterial infection primarily affecting the small intestine.
- Characterized by accumulation of periodic acid-Schiff-positive material in macrophages.
- Initial treatment involves antibiotics, but relapses can occur with multi-organ involvement.
Observation:
- A 4-year follow-up of a Whipple's disease patient revealed persistent impairment in monocyte and macrophage bacterial degradation.
- Relapse was associated with reduced duodenal brush border enzyme activity.
- Flow cytometry demonstrated ongoing cellular immune dysfunction throughout the study period.
Findings:
- Despite antibiotic treatment (oxytetracycline, chloramphenicol, trimethoprim-sulfamethoxazole), intracellular bacterial degradation by phagocytes remained impaired.
- Complete clinical and histopathologic remission, along with restoration of duodenal enzyme activity, was achieved after 17 months of continuous trimethoprim-sulfamethoxazole.
- The study identified a persistent mononuclear phagocyte dysfunction in the patient.
Implications:
- Suggests a potential primary defect in cell-mediated immunity in Whipple's disease.
- This immune dysfunction may predispose individuals to infection by the causative bacillus.
- Highlights the importance of long-term therapeutic strategies and monitoring immune function in Whipple's disease management.