TY - JOUR
T1 - Whipple disease a century after the initial description
T2 - Increased recognition of unusual presentations, autoimmune comorbidities, and therapy effects
AU - Arnold, Christina A.
AU - Moreira, Roger K.
AU - Lam-Himlin, Dora
AU - De Petris, Giovanni
AU - Montgomery, Elizabeth
PY - 2012/7
Y1 - 2012/7
N2 - Although Whipple disease was described over a century ago, it remains challenging to recognize. To better understand the presentation of Whipple disease, we undertook a clinicopathologic study of our experience since implementation of the Whipple immunohistochemical stain. Twenty-three biopsy specimens from 15 patients were identified, and an association with immunomodulatory conditions was noted. Whipple disease involved the small intestine (19), brain (2), breast (1), and retroperitoneum (1). Whipple disease was suspected by 3 clinicians and by the majority of pathologists (9). Alternative clinical impressions included lymphoma, celiac disease, Crohn vasculitis, sepsis, an inflammatory process, liposarcoma, rheumatoid arthritis, seizure disorder, cerebrovascular accident, xanthoma, and central nervous system neoplasm. The nonspecific nature of the disease presentation likely contributed to the extended period between onset of symptoms and a definitive diagnosis, which ranged from at least 1 year to over 10 years. One patient died of unknown causes, and both patients with detailed follow-up had clinically persistent disease. We also describe Whipple disease with therapy effects, including partial and complete histologic treatment effects. Awareness of the unusual clinicopathologic presentations of Whipple disease is essential for timely diagnosis of this potentially lethal disease.
AB - Although Whipple disease was described over a century ago, it remains challenging to recognize. To better understand the presentation of Whipple disease, we undertook a clinicopathologic study of our experience since implementation of the Whipple immunohistochemical stain. Twenty-three biopsy specimens from 15 patients were identified, and an association with immunomodulatory conditions was noted. Whipple disease involved the small intestine (19), brain (2), breast (1), and retroperitoneum (1). Whipple disease was suspected by 3 clinicians and by the majority of pathologists (9). Alternative clinical impressions included lymphoma, celiac disease, Crohn vasculitis, sepsis, an inflammatory process, liposarcoma, rheumatoid arthritis, seizure disorder, cerebrovascular accident, xanthoma, and central nervous system neoplasm. The nonspecific nature of the disease presentation likely contributed to the extended period between onset of symptoms and a definitive diagnosis, which ranged from at least 1 year to over 10 years. One patient died of unknown causes, and both patients with detailed follow-up had clinically persistent disease. We also describe Whipple disease with therapy effects, including partial and complete histologic treatment effects. Awareness of the unusual clinicopathologic presentations of Whipple disease is essential for timely diagnosis of this potentially lethal disease.
KW - Tropheryma whipplei
KW - Whipple disease
KW - immunohistochemistry
KW - partial and complete histologic treatment effect
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U2 - 10.1097/PAS.0b013e31825a2fa4
DO - 10.1097/PAS.0b013e31825a2fa4
M3 - Article
C2 - 22743287
AN - SCOPUS:84863302867
SN - 0147-5185
VL - 36
SP - 1066
EP - 1073
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
IS - 7
ER -