Intestinal Lymphangiectasia With Protein-Losing Enteropathy in Waldenstrom Macroglobulinemia.

Intestinal Lymphangiectasia With Protein-Losing Enteropathy in Waldenstrom Macroglobulinemia.

Medicine (Baltimore). 2007 Jul

Pratz KW, Dingli D, Smyrk TC, Lust JA.
From Department of Medical Oncology (KWP), Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Hospital, Baltimore, Maryland; Division of Hematology (DD, JAL), Department of Internal Medicine; and Department of Laboratory Medicine and Pathology (TCS), Mayo Clinic College of Medicine, Rochester, Minnesota.

Gastrointestinal complications of Waldenstrom macroglobulinemia (WM) are unusual but often treatable. We report a case of WM associated with significant gastrointestinal involvement manifest as chronic diarrhea with protein-losing enteropathy and recurrent venous thromboses. Small bowel biopsy was negative for amyloidosis but revealed intestinal lymphangiectasia with deposition of monoclonal IgM. The patient was treated with cyclophosphamide, vincristine, and prednisone with rapid and complete resolution of the peripheral edema and diarrhea. We follow the case report with a retrospective analysis of patients with WM and gastrointestinal symptoms seen at our institution, and review the available literature on this unusual association.

An increased awareness of the gastrointestinal manifestations of WM may help to explain and to treat the chronic, debilitating, and potentially life-threatening symptoms in patients with this lymphoproliferative disorder.

Lippincott, Williams & Wilkins

Clinical application and diagnostic yield of wireless capsule endoscopy in children

Clinical application and diagnostic yield of wireless capsule endoscopy in children

J Laparoendosc Adv Surg Tech A. 2007 Jun

Antao B, Bishop J, Shawis R, Thomson M.
Pediatric Surgical Unit and Sheffield Children's Hospital, Sheffield, United Kingdom.

Objective:

The small bowel is anatomically difficult to examine and was investigated by invasive, indirect modalities, such as push enteroscopy and small bowel follow-through. The aim of this study was to assess the efficacy and clinical impact of wireless capsule endoscopy (WCE) in children.

Materials and Methods:

Over the last 3 years (2002-2005), 37 patients with suspected small-bowel disease were investigated with WCE at a median age of 11 years (range, 16 months-16 years). The indications for WCE was suspected Crohn's disease (CD) (18), obscure or occult gastrointestinal bleeding (7), polyposis syndromes (5), protein losing enteropathy (4), recurrent abdominal pain (2), and malabsorption syndrome (1). All patients had preceding upper gastrointestinal endoscopy (OGD), ileocolonoscopy, and 26 cases had a small bowel follow-through (SBFT). These results were compared with the findings on WCE.

Results:

Thirty-three (33) cases successfully completed the WCE through the small bowel. Four (4) patients were unable to swallow the capsule, 3 of which had to be placed in the duodenum endoscopically. In 3 patients, the capsule remained in the stomach and no small bowel images were obtained. The overall diagnostic yield was 85% (28/33 patients). The diagnostic findings included CD (13), source of gastrointestinal bleeding (7), polyposis syndromes (3), erosive enteropathy and patchy lymphangiectasia (4), and intussusception (1). WCE was found to be more sensitive for small bowel pathology than SBFT (20 vs. 6 [30% sensitivity, compared to WCE]) and endoscopic investigations (28 vs. 12 [43% sensitivity compared to WCE]). As a result of WCE findings, there was a positive alteration in the management in 28 of 33 (85%) cases.

Conclusions:

WCE is a novel, noninvasive, and useful tool for the investigation of the small intestine in children. It is superior and more sensitive than other conventional endoscopic and radiologic investigations in the assessment of the small bowel. It can help in guiding surgical decisions and should be routinely integrated as a part of the diagnostic work-up of small bowel pathology.

Article

Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome) with lymphangiectasia

Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome).
With lymphangiectasia as a complication

Diagn Pathol. 2007 Jul

Vankalakunti M, Gupta K, Kakkar N, Das A.

ABSTRACT
BACKGROUND: Renal-Hepatic-Pancreatic dysplasia syndrome described by Ivemark in 1959 constitutes a triad pancreatic fibrosis, renal dysplasia and hepatic dysgenesis.

CASE PRESENTATION: We describe two unrelated cases of Renal-hepatic-pancreatic dysplasia syndrome in stillborn babies. The characteristic microscopic features were present in both the cases. The second case illustrates the unique association lymphangiectasia with Renal-hepatic-pancreatic dysplasia syndrome. Both cases are unrelated and there is no history of any consanguineous marriage.

CONCLUSION: These two cases are unrelated and are rare. In the developmental research, the perinatal autopsy needs to be utilized as a major tool and an Ad hoc committee formation is required to formulate the approach towards syndromic diseases.

Diagnostic Pathology