Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania

Abstract

The patients were categorized in three subgroups of IPSID: alpha-chain disease (n=8), non-alpha chain disease with other monoclonal immunoglobulins (n=3), and polyclonal 'non-malignant' IPSID (n=2). In several patients the disease had unusual features, and this in some cases delayed the diagnosis. In suspected cases it is thus of the utmost importance to proceed to an exploratory laparatomy. Patients with stage C disease had a short survival, whereas two patients with stage A alpha-chain disease responded to treatment with cyclophosphamide, vincristine and prednisolone, and cyclophosphamide, doxorubicine, vincristine and prednisolone, respectively, have a disease-free long survival of 35 and 12 years, and appear to be cured.

Emergency Pancreatoduodenectomy (Whipple Procedure) for Massive Upper Gastrointestinal Bleeding Caused by a Diffuse B-Cell Lymphoma of the Duodenum: Report of a Case

“…However, initially benign-appearing lesions often evolve into aggressive high-grade lymphomas (as in our case), invading the intestinal wall and mesenteric lymph nodes, and they may also metastasize to distant organs. 3,4 In half of the cases, the initially diagnosed IPSID coexists with a high-grade B-cell lymphoma. 1 The fi rst-line therapy of the advanced stage of IPSID is rituximab (a chimeric monoclonal antibody directed against the CD20 antigen), which, in combination with conventional chemotherapy, has been associated with higher response rates, a longer event-free survival, and a longer overall survival, over combination chemotherapy alone.…”

“…Molecular and immunohistochemical studies have demonstrated an association with Helicobacter pylori and Campylobacter jejuni. 3,4 The symptoms include diarrhea, abdominal pain, weight loss, and occasionally fever and lymphadenopathy. 3,4 A staging system for IPSID has emerged as a reliable prognostic and therapeutic parameter.…”

“…3,4 The symptoms include diarrhea, abdominal pain, weight loss, and occasionally fever and lymphadenopathy. 3,4 A staging system for IPSID has emerged as a reliable prognostic and therapeutic parameter. Stage A is characterized by a mature plasmacytic or lymphoplasmacytic infi ltration of the lamina propria.…”

“…Although this disorder mostly affects the young population of low socioeconomic class in developing countries (Middle East and Africa), a number of cases have emerged also in developed countries. 3 This condition is characterized by a dense lymphoplasmacytic infi ltrate beneath the epithelium of the duodenal and the proximal jejunal mucosa and also in the mesenteric lymph nodes. The B-lymphocytes involved elaborate an intracellular synthesis of an abnormal immunoglobulin (alpha heavy chain).…”

Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)

“…IPSID has had many synonyms due to its broad symptomatic spectrum. In the early 1960s, young adults from Israel were reported to present small intestinal lymphoma associated with malabsorption symptoms, coining the term Mediterranean Lymphoma to this disease, as it was predominantly found in patients from that geographical region [1,2]. In 1965, Bracha was the first to describe 13 patients in Tel-Hashomer hospital with this syndrome [3,4].…”