Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts.

Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts.

Gut. 2000 Aug

Bouhnik Y, Etienney I, Nemeth J, Thevenot T, Lavergne-Slove A, Matuchansky C.
Department of Gastroenterology and Hepatology, Hôpital Lariboisière, Paris, France. yoram.bouhnik@lrb.ap-hop-paris.fr

As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this association is merely fortuitous or related to primary intestinal lymphangiectasia induced immune deficiency. We report on two female patients, 50 and 58 years old, who developed small intestinal high grade B cell lymphoma a long time (45 and 40 years, respectively) after the initial clinical manifestations of primary intestinal lymphangiectasia. They presented with a longstanding history of fluctuating protein losing enteropathy, multiple cutaneous plane warts, and markedly dilated mucosal and submucosal lymphatic channels in duodenal biopsies. One had a large ulcerated tumour of the proximal ileum and the other diffuse ileal infiltration. In both, histological examination showed centroblastic high grade B cell lymphoma associated with duodenojejuno-ileal mucosal and submucosal lymphangiectasia. They were subsequently successfully treated with surgery and postoperative chemotherapy (AVmCP: adriamycin, cyclophosphamide, Vm26, and prednisolone), and chemotherapy alone (PACOB: adriamycin, cyclophosphamide, vincristine, bleomycine, and prednisolone), respectively. A three year follow up in both cases showed persistent diffuse lymphangiectasia without evidence of lymphoma. The present findings support the hypothesis that primary intestinal lymphangiectasia is associated with lymphoma development.

Gut Online

Protein-losing enteropathy in a case of nodal follicular lymphoma without a gastrointestinal mucosal lesion.

Protein-losing enteropathy in a case of nodal follicular lymphoma without a gastrointestinal mucosal lesion.
Intern Med. 2008
Kaneko H, Yamashita M, Ohshiro M, Ohkawara Y, Matsumoto Y, Nomura K, Horiike S, Yokota S, Taniwaki M.
Department of Hematology, Aiseikai-Yamashina Hospital, Kyoto. hp@aiseikaihp.or.jp

Protein-losing enteropathy (PLE) is characterized by gastrointestinal loss of serum protein. It is usually caused by hypersecretion from a tumor, ulcer, or long standing lymphangiectasia. However, we report a 47-year-old man of peritoneal nodal follicular lymphoma who developed PLE with none of them. Oozing of whitish fluid from duodenal bulbar mucosa was endoscopically seen, resulting in continuous loss of protein. Chemotherapy was effective and PLE was rapidly diminished. Nodal lymphoma lesion was considered to disturb lymphatic flow and to regurgitate it to duodenal mucosa. To our knowledge, this is the second report of a lymphoma patient presenting PLE without a gastrointestinal mucosal lesion.

Internal Medicine

Intestinal lymphangiectasia secondary to radiotherapy and chemotherapy.

This is an older article going back to 1987. I found it interesting however as it is a report of secondary intestinal lymphangiectasia from an unexpected souce, cancer treatment.

The article:

Intestinal lymphangiectasia secondary to radiotherapy and chemotherapy.

Dig Dis Sci. 1987 Aug

Rao SS,
Dundas S,
Holdsworth CD.

We report a case of intestinal lymphangiectasia secondary to radiotherapy and chemotherapy. The patient also had small bowel bacterial overgrowth and pancreatic insufficiency. Lymphatic ectasia as a histological feature has been described previously in association with postradiotherapy malabsorption, but radiation-induced lymphangiectasia producing clinical manifestations has hitherto not been reported. Replacement of dietary long-chain fats with medium-chain triglycerides, pancreatic enzyme supplements, and a short course of oxytetracycline, resulted in dramatic clinical improvement.

The possibility of intestinal lymphangiectasia should be borne in mind in patients with postradiotherapy malabsorption. A low serum albumin and lymphocyte count should draw attention to this possibility.

PMID: 3608736 [PubMed - indexed for MEDLINE]