Sunday 20 January 2008
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.
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.
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.
Although these structures are not fungi, the GMS impregnates macrophage vacuoles boldly. Most are displayed as amorphous or globoid bodies.
Tropheryma whipplei-associated granulomatous myelitis
Tropheryma whipplei can be detected in the bone marrow trephine by polymerase chain reaction.
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