What is Whipple's disease?

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From the Guidelines

Whipple's disease is not a disease, but rather a surgical procedure.

Definition of Whipple's Procedure

It is also known as a pancreatoduodenectomy, which is a surgical operation that involves the removal of the head of the pancreas, the duodenum, the gallbladder, and a portion of the bile duct [ 1 ].

Indications for the Procedure

The Whipple procedure is typically performed to treat pancreatic cancer that is located in the head of the pancreas [ 1 ].

Key Steps in the Procedure

The procedure involves several key steps, including:

  • Dissection of the right hemi-circumference of the superior mesenteric artery (SMA) to the right of the coeliac trunk [ 1 ]
  • Removal of the head of the pancreas, the duodenum, and the gallbladder
  • Reconstruction of the gastrointestinal tract and the bile duct

Goals of the Procedure

The primary goal of the Whipple procedure is to achieve an R0 resection, which means that all of the cancerous tissue is removed, and there are no cancer cells left behind [ 1 ].

Potential Complications

The Whipple procedure is a complex and major surgical operation that carries significant risks and potential complications, including infection, bleeding, and delayed gastric emptying [ 1 ].

From the Research

Definition and Cause of Whipple's Disease

  • Whipple's disease is a chronic, systemic infection caused by Tropheryma whipplei 2, 3, 4, 5, 6.
  • It is a rare, multisystemic infectious disorder that affects predominantly middle-aged men 3, 6.
  • The disease is caused by the bacterium Tropheryma whipplei, which is a ubiquitous commensal bacterium 2.

Clinical Manifestations

  • The disease presents with a variety of clinical manifestations, including weight loss, arthralgia, diarrhea, and abdominal pain 3, 4, 5, 6.
  • Other symptoms may include fever, hyperpigmentation, and lymphadenopathy 4, 5.
  • The disease can affect various organs, including the small intestine, central nervous system, joints, and eyes 2, 3, 4, 5, 6.

Diagnosis

  • The diagnosis of Whipple's disease is made by biopsy of the small intestine mucosa, which reveals infiltration of the lamina propria with periodic acid-Schiff positive macrophages 4, 5, 6.
  • Other diagnostic tests include polymerase chain reaction (PCR) of the 16S ribosomal RNA of Tropheryma whipplei, electron microscopy, and immunohistochemistry 2, 3, 4, 6.
  • A small bowel biopsy is often diagnostic, but in some cases, a stereotactic brain biopsy may be necessary 4.

Treatment and Prognosis

  • The treatment of Whipple's disease involves antibiotic therapy, typically with trimethoprim-sulfamethoxazole or a third-generation cephalosporin 2, 4, 5, 6.
  • The response to treatment should be monitored closely, as relapses are common 4, 6.
  • If diagnosed and treated early, the prognosis is excellent, but if left untreated, the disease can be fatal 2, 3, 4, 5, 6.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Whipple's disease.

Annals of the rheumatic diseases, 2013

Research

Whipple's disease.

Journal of the neurological sciences, 2017

Research

Whipple's disease.

Postgraduate medical journal, 2000

Research

Whipple's disease.

Digestive diseases (Basel, Switzerland), 1995

Research

Whipple's disease.

Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association, 2004

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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