“Mediterranean abdominal lymphoma,” or immunoproliferative small intestinal disease.Part I: Clinical aspects

Abstract

Twenty‐five cases of “Mediterranean abdominal lymphoma,” better known as immunoproliferative small intestinal disease (IPSID), are reported from the American University of Beirut Hospital. All patients were Moslem Arabs from low socio‐economical background. The mean age was 25 years. IPSID is shown in this study to be a distinct disease entity characterized by a triad of clinical, pathological and immunoglobulin abnormalities. The clinical manifestations of the disease included chronic diarrhea, weight loss, diffuse abdominal pain, clubbing of fingers and toes and occasionally palpable peripheral lymph nodes. Radiological studies revealed a malabsorption pattern with evidence of mucosal fold thickening, segmentation and flocculation of barium. Laboratory investigations showed evidence of malabsorption, hypoalbuminemia and hypocalcemia. Two patients lacked evidence of malabsorption. Nine patients had alpha heavy chain protein in the serum. However, intestinal fluid immunoelectropheresis and immunofluorescence studies on involved intestinal mucosa were not performed and therefore, alpha heavy chain disease (αHCD) could not be excluded in the remaining 16 patients. A new staging classification is proposed, and the role of laparotomy in staging is emphasized. In five patients, mesenteric lymph nodes harboured immunoblastic sarcoma whereas the intestinal mucosa in the same patients was involved with a benign‐appearing lymphoplasmacytic cellular infiltrate without evidence of malignancy. Three patients had histologically proven lymphoma in peripheral lymph nodes and all had αHCD. Lymphomatous involvement of distant organs was not observed. One patient with αHCD was considered in the premalignant phase of IPSID. Prognosis was poor as the majority of patients succumbed within two years after diagnosis. The significance of early detection is emphasized. Cancer 40:2941‐2947, 1977.

Non‐hodgkin's lymphomas in Jordanians: A histopathological study of 231 cases

“…It is interesting to note that only four Jordanian cases with abdominal, diffuse histiocytic lymphoma showed clinical and morphological features consistent with the Mediterranean abdominal lymphoma previously described (Rappaport et af., 1972;Al-Saleem and Al-Bahrani, 1973;Doe, 1975;WHO Report, 1975;Salem et al, 1977;Nassar et af., 1978). It appears that this type of lymphoma may be less frequent than previously thought.…”

Five-year results of the treatment of 23 patients with immunoproliferative small intestinal disease

“…Five deaths due to disease progression were obdiarrhea and malabsorption in patients with IPSID. 8,13 Although the epidemiologic features of the disease and served in the current study. Treatment toxicities were in acceptable limits.…”

Improved Survival of Children with Non-Hodgkin's Lymphoma with Intermediate Dose Methotrexate, Intrathecal Methotrexate, and Citrovorum Factor Rescue

“…5 Immunoproliferative small intestinal disease (IPSID), or Mediterranean lymphoma, was added to this classification because it is relevant to the Middle East. 12 Clinical staging was done according to Murphy's staging system. 13 This, however, was of little practical value because all patients in this series except one had locally advanced or disseminated disease at the time of diagnosis.…”