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

As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this associatio n is merely fortuitous or related to primary intestinal lymphangiectasia in duced 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 o f primary intestinal lymphangiectasia. They presented with a longstanding h istory of fluctuating protein losing enteropathy, multiple cutaneous plane warts, and markedly dilated mucosal and submucosal lymphatic channels in du odenal biopsies. One had a large ulcerated tumour of the proximal ileum and the other diffuse heal infiltration. In both, histological examination sho wed centroblastic high grade B cell lymphoma associated with duodenojejuno- ileal mucosal and submucosal lymphangiectasia. They were subsequently succe ssfully treated with surgery and postoperative chemotherapy (AVmCP: adriamy cin, cyclophosphamide, Vm26, and prednisolone), and chemotherapy alone (PAC OB: adriamycin, cyclophosphamide, vincristine, bleomycine, and prednisolone ), respectively. A three year follow up in both cases showed persistent dif fuse lymphangiectasia without evidence of lymphoma. The present findings su pport the hypothesis that primary intestinal lymphangiectasia is associated with lymphoma development.