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Whipple disease
Sunday 20 January 2008
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Whipple’s Disease by Ed Euthman
Definition: Whipple’s disease (WD) is a chronic systemic inflammatory disease of infectious origin caused by Tropheryma whipplei (TW).
Abdominal pain and recurrent diarrhea are usually the main symptoms leading to the suspicion of a primary bowel disease. Systemic manifestations can mimic hematologic disorders.
Microscopy
The duodenal mucosa shows villi that are distended with large pink cells.
The deep portion of the mucosa is relatively unaffected.
The lamina propria contains swollen histiocytes and large lipid vacuoles.
Differential diagnosis
The most important item on the differential diagnosis is mycobacterial infection in an immunocompromised (usually AIDS) patient.
In a mycobacterial infection, these enlarged histiocytes are stuffed with innumerable acid-fast bacilli.
The GMS has to be performed to rule out histoplasmosis and other fungal infections.
Although these structures are not fungi, the GMS impregnates macrophage vacuoles boldly. Most are displayed as amorphous or globoid bodies.
Clinical synopsis
fever
weight loss
long-standing arthralgia
diarrhea
Synopsis
Tropheryma whipplei-associated granulomatous myelitis
Diagnosis
Tropheryma whipplei can be detected in the bone marrow trephine by polymerase chain reaction.
See also
granulomatous myelitis
References
Whipple disease a century after the initial description: increased recognition of unusual presentations, autoimmune comorbidities, and therapy effects. Arnold CA, Moreira RK, Lam-Himlin D, De Petris G, Montgomery E. Am J Surg Pathol. 2012 Jul;36(7):1066-73. PMID: 22743287
Fenollar F, Puéchal X, Raoult D. Whipple’s disease. N Engl J Med. 2007 Jan 4;356(1):55-66. PMID: 17202456
Primary diagnosis of Whipple’s disease in bone marrow. Kröber SM, Kaiserling E, Horny HP, Weber A. Hum Pathol. 2004 Apr;35(4):522-5. PMID: 15116337
