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Abstract:

Classical Whipple’s disease is usually diagnosed by periodic acid-Schiff staining of small-bowel biopsy specimens. These show typical infiltrations of the lamina propria with macrophages containing granular inclusions which represent intracellular or ingested bacteria. PCRs to detect DNA in clinical samples are of growing importance to confirm histological results. Whipple’s disease requires long-term antibiotic treatment. In the case of relapse and failure of co-trimoxazole treatment, therapy with doxycycline plus hydroxychloroquine is an effective alternative. This chapter summarizes the latest findings on the epidemiology, biology, pathogenicity, and immunological control of this fastidious Gram-positive bacterium. One focus is on the clinical appearance, which is crucial for the initial suspicion of Whipple’s disease, the decision to initiate diagnostic tests, and the assessment of laboratory results. The chapter also covers the laboratory methods, presents a hierarchical scheme for the diagnosis of Whipple’s disease, and discusses the current treatment options.

Citation: GeißdÖrfer W, Moter A, Bogdan C. 2015. , p 1159-1168. In Jorgensen J, Pfaller M, Carroll K, Funke G, Landry M, Richter S, Warnock D (ed), Manual of Clinical Microbiology, Eleventh Edition. ASM Press, Washington, DC. doi: 10.1128/9781555817381.ch67
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Figures

Image of FIGURE 1
FIGURE 1

PAS staining of a duodenal biopsy specimen shows lymphangiectasia and massive infiltration of the lamina propria by strongly PAS-positive foamy macrophages containing (image kindly provided by Christoph Loddenkemper, Pathotres, Berlin, Germany). doi:10.1128/9781555817381.ch67.f1

Citation: GeißdÖrfer W, Moter A, Bogdan C. 2015. , p 1159-1168. In Jorgensen J, Pfaller M, Carroll K, Funke G, Landry M, Richter S, Warnock D (ed), Manual of Clinical Microbiology, Eleventh Edition. ASM Press, Washington, DC. doi: 10.1128/9781555817381.ch67
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Image of FIGURE 2
FIGURE 2

FISH of a duodenal biopsy of an untreated Whipple’s disease patient using a -specific probe ( ). The low-magnification image shows clusters of cells (yellow) against the slightly greenish autofluorescent background of the tissue. Host cell nuclei are stained blue with 4′, 6-diamidino-2-phenylindole (DAPI). Inset shows single bacteria at a higher magnification. doi:10.1128/9781555817381.ch67.f2

Citation: GeißdÖrfer W, Moter A, Bogdan C. 2015. , p 1159-1168. In Jorgensen J, Pfaller M, Carroll K, Funke G, Landry M, Richter S, Warnock D (ed), Manual of Clinical Microbiology, Eleventh Edition. ASM Press, Washington, DC. doi: 10.1128/9781555817381.ch67
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Image of FIGURE 3
FIGURE 3

FISH of a heart valve section in a case of culture-negative endocarditis using a -specific probe (Cy3, orange) ( ). The low-magnification image shows extensive areas with bacterial colonization (slightly orange glimmer) along with calcified tissue (bright yellow-green autofluorescence). Host cell nuclei are stained blue with 4′,6-diamidino-2-phenylindole (DAPI). Inset shows dense clusters of cells at a higher magnification (orange). doi:10.1128/9781555817381.ch67.f3

Citation: GeißdÖrfer W, Moter A, Bogdan C. 2015. , p 1159-1168. In Jorgensen J, Pfaller M, Carroll K, Funke G, Landry M, Richter S, Warnock D (ed), Manual of Clinical Microbiology, Eleventh Edition. ASM Press, Washington, DC. doi: 10.1128/9781555817381.ch67
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Image of FIGURE 4
FIGURE 4

Hierarchical scheme for the diagnosis of Whipple’s disease (modified from and ). 1, positive PCR should be confirmed by 16S rRNA gene sequencing or a second specific PCR detecting a different target sequence; 2, IHC and FISH are available only in specialized laboratories. doi:10.1128/9781555817381.ch67.f4

Citation: GeißdÖrfer W, Moter A, Bogdan C. 2015. , p 1159-1168. In Jorgensen J, Pfaller M, Carroll K, Funke G, Landry M, Richter S, Warnock D (ed), Manual of Clinical Microbiology, Eleventh Edition. ASM Press, Washington, DC. doi: 10.1128/9781555817381.ch67
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Tables

Generic image for table
TABLE 1

Symptoms and signs of infections

Citation: GeißdÖrfer W, Moter A, Bogdan C. 2015. , p 1159-1168. In Jorgensen J, Pfaller M, Carroll K, Funke G, Landry M, Richter S, Warnock D (ed), Manual of Clinical Microbiology, Eleventh Edition. ASM Press, Washington, DC. doi: 10.1128/9781555817381.ch67
Generic image for table
TABLE 2

Recommended therapy for Whipple’s disease ( )

Citation: GeißdÖrfer W, Moter A, Bogdan C. 2015. , p 1159-1168. In Jorgensen J, Pfaller M, Carroll K, Funke G, Landry M, Richter S, Warnock D (ed), Manual of Clinical Microbiology, Eleventh Edition. ASM Press, Washington, DC. doi: 10.1128/9781555817381.ch67

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