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Whipple's disease
WHIPPLE’S DISEASE
A 49-year-old man was hospitalized because of diarrhea, malnutrition, and weight loss. Generalized body wasting was readily evident, together with ascites and peripheral edema. Laboratory test results included the following: Hb, 6 g/dL (normal: 1417, 5 g/dL); total protein, 4.9 g/dL (6-8 g/dL); prothrombin time, 61%; serum iron, 6.8 mcmol/L (11.6-31.3 mcmol/L); and total cholesterol, 93 mg/dL (100-239 mg/dL). The C-reactive protein level was markedly elevated. The serum ferratin level was normal, and stool cultures were negative, as were tests for Mycobacterium tuberculosis, hepatitis A virus, hepatitis B virus, and hepatitis C virus. Transabdominal US disclosed an enlarged liver and free fluid in the peritoneal cavity. Because the patient had been drinking large amounts of alcohol, had lost his job, and was homeless, protein-calorie malnutrition was suspected. However, the anemia 800
GASTROINTESTINAL ENDOSCOPY
was only partially corrected by blood transfusions. Lymphocyte subpopulations were normal. Fecal alpha-1-antitripsin, fecal occult blood tests, folic acid and vitamin B12 levels, permeability to lactulose/ mannitol, and tests for antitransglutaminase and cytomegalovirus (CMV) and HIV antibodies were all normal or negative. Upper endoscopy and duodenal biopsy specimens were negative. Retroperitoneal and mesenteric lymphadenopathy, ascites, and thickened bowel loops were evident on CT. Barium contrast radiography demonstrated only hypotonic, dilated loops of small bowel. Capsule endoscopy revealed enlarged, hypertrophic villi, with intervening areas of denuded mucosa, together with blood clots and petechiae in the proximal jejunum (A and B). Multiple whitish plaque-like patches covered the villi throughout the jejunum and ileum. On histopathologic evaluation of multiple biopsy specimens VOLUME 60, NO. 5, 2004
At the Focal Point...
obtained at enteroscopy, the lamina propria was expanded by a diffuse infiltrate of large, pale macrophages (C; H&E, orig. mag. 3200) that contained cytoplasmic granular particles, which stained positive with periodic-acid-Schiff stain (D; orig. mag. 3200). Tropheryma whippelii DNA was demonstrated by polymerase chain reaction. The final diagnosis was Whipple’s disease. Renata D’Inca`, MD Department of Surgical and Gastroenterological Sciences Antonio Pagnan, MD Division of Internal Medicine
Maria Grazia Vettorato, RN Department of Surgical and Gastroenterological Sciences Giuseppe Ingravallo, MD Massimo Rugge, MD Department of Pathology Giacomo Carlo Sturniolo, MD Department of Surgical and Gastroenterological Sciences University of Padua Padua, Italy
PII: S0016-5107(04)02043-7
PROTEIN-LOSING ENTEROPATHY
A 31-year-old man was referred because of bilateral ankle edema and ascites from hypoalbuminemia. At age 17 years, he was found to have a retroperitoneal mass, the nature of which was never established. CT revealed a 10 cm, lowattenuation mesenteric mass (A). Leakage of proVOLUME 60, NO. 5, 2004
tein from the small and the large bowel was confirmed by radioisotope-labeled-albumin scintigraphy. Endoscopically, the duodenal folds were prominent (B) and, by EUS, contained dilated, mucosal, vessel-like channels (C). In addition, EUS demonstrated the retroperitoneal mass to be GASTROINTESTINAL ENDOSCOPY
801
A 49-year-old man was hospitalized because of diarrhea, malnutrition, and weight loss. Generalized body wasting was readily evident, together with ascites and peripheral edema. Laboratory test results included the following: Hb, 6 g/dL (normal: 1417, 5 g/dL); total protein, 4.9 g/dL (6-8 g/dL); prothrombin time, 61%; serum iron, 6.8 mcmol/L (11.6-31.3 mcmol/L); and total cholesterol, 93 mg/dL (100-239 mg/dL). The C-reactive protein level was markedly elevated. The serum ferratin level was normal, and stool cultures were negative, as were tests for Mycobacterium tuberculosis, hepatitis A virus, hepatitis B virus, and hepatitis C virus. Transabdominal US disclosed an enlarged liver and free fluid in the peritoneal cavity. Because the patient had been drinking large amounts of alcohol, had lost his job, and was homeless, protein-calorie malnutrition was suspected. However, the anemia 800
GASTROINTESTINAL ENDOSCOPY
was only partially corrected by blood transfusions. Lymphocyte subpopulations were normal. Fecal alpha-1-antitripsin, fecal occult blood tests, folic acid and vitamin B12 levels, permeability to lactulose/ mannitol, and tests for antitransglutaminase and cytomegalovirus (CMV) and HIV antibodies were all normal or negative. Upper endoscopy and duodenal biopsy specimens were negative. Retroperitoneal and mesenteric lymphadenopathy, ascites, and thickened bowel loops were evident on CT. Barium contrast radiography demonstrated only hypotonic, dilated loops of small bowel. Capsule endoscopy revealed enlarged, hypertrophic villi, with intervening areas of denuded mucosa, together with blood clots and petechiae in the proximal jejunum (A and B). Multiple whitish plaque-like patches covered the villi throughout the jejunum and ileum. On histopathologic evaluation of multiple biopsy specimens VOLUME 60, NO. 5, 2004
At the Focal Point...
obtained at enteroscopy, the lamina propria was expanded by a diffuse infiltrate of large, pale macrophages (C; H&E, orig. mag. 3200) that contained cytoplasmic granular particles, which stained positive with periodic-acid-Schiff stain (D; orig. mag. 3200). Tropheryma whippelii DNA was demonstrated by polymerase chain reaction. The final diagnosis was Whipple’s disease. Renata D’Inca`, MD Department of Surgical and Gastroenterological Sciences Antonio Pagnan, MD Division of Internal Medicine
Maria Grazia Vettorato, RN Department of Surgical and Gastroenterological Sciences Giuseppe Ingravallo, MD Massimo Rugge, MD Department of Pathology Giacomo Carlo Sturniolo, MD Department of Surgical and Gastroenterological Sciences University of Padua Padua, Italy
PII: S0016-5107(04)02043-7
PROTEIN-LOSING ENTEROPATHY
A 31-year-old man was referred because of bilateral ankle edema and ascites from hypoalbuminemia. At age 17 years, he was found to have a retroperitoneal mass, the nature of which was never established. CT revealed a 10 cm, lowattenuation mesenteric mass (A). Leakage of proVOLUME 60, NO. 5, 2004
tein from the small and the large bowel was confirmed by radioisotope-labeled-albumin scintigraphy. Endoscopically, the duodenal folds were prominent (B) and, by EUS, contained dilated, mucosal, vessel-like channels (C). In addition, EUS demonstrated the retroperitoneal mass to be GASTROINTESTINAL ENDOSCOPY
801