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.

The pathology of 25 cases of Mediterranean abdominal lymphoma, better designated as immunoproliferative small intestinal disease (IPSID), are reported from the American University of Beirut Hospital. The series includes nine cases with documented alpha heavy chain disease (alpha-HCD). The disease is characterized by the presence of a diffuse and compact bandlike lymphoplasmacytic infiltration of the proximal small intestinal mucosa. The presence of a concomitant malignant lymphoma in the intestine and/or mesenteric lymph nodes, and of alpha-heavy protein in the serum is commonly encountered. Two histopathologic variants of IPSID are present. The first is characterized by the diffuse infiltration of the mucosa, at sites away from tumoral masses, by either pure plasmacytic infiltration, or mixed lymphoplasmacytic infiltration. This variety is associated with the immunoblastic sarcoma type of malignant lymphoma, and with alpha chain disease (alpha-HCD). The second variant is characterized by a diffuse follicular lymphoid hyperplasia pattern in the small intestinal mucosa. The associated malignant lymphoma is diffuse and undifferentiated often having a starry-sky pattern. This variety is not associated with alpha-HCD. Both histologic variants share the same clinical antecedents. In five patients, mesenteric lymph nodes harbored immunoblastic sarcoma while the intestinal mucosae of the same patients were involved with a benign appearing lymphoplasmacytic infiltration. This finding stresses the need for staging laparatomy. Three patients, with alpha-HCD, had peripheral lymph node involvement with immunoblastic sarcoma. The disease apparently evolves in two stages: an immunoproliferative phase, probably reversible, and a later development of malignant lymphoma. The term immunoproliferative small intestinal disease accurately describes the nature of the entity.

THE STUDY of selective factors responsible for the maintenance of the thalassaemic gene in a well-defined population has been the subject of interest to several investigators in the last decade. It is thought that, as a result of certain biological advantages, a greater number of thalassaemic heterozygotes survive into reproductive life thus compensating for the continuous loss of the homozygous form of the disease.The first biological advantage in this balanced polymorphism proposed by Haldane (1945) was malarial infection. According to this author individuals harbouring the abnormal thalassacmic gene were found to be more resistant against invasion by the parasite. Studies on the incidence of thalassaemia in populations with a past or present history of malaria by Carcassi, Ceppellini and Pitzus (1957) have given support to Haldane's hypothesis. Recently Stamatoyannopoulos and Fessas (1964) found that, compared to the sickle-cell disease and G6PD deficiency, thalassaemia is the least efficient abnormality protecting against malaria. Further investigations on this relationship are at present being pursued.The second selective biological advantage is based on a totally di&=rent mechanism and was proposed by Sijpesteijn (1958). This investigator suggested that the heterozygote thalassaemic enjoyed a greater protection against iron deficiency than the normal individual. The mild chronic anaemia in women affected with this abnormal gene might enhance iron absorption with increased storage in the tissues. This hypothesis could not be confirmed by several Italian investigators. Rannerman and Callender (1961) have studied iron absorption in 17 heterozygote thalassaemics using haemoglobin labelled with 59Fe and inorganic 5 9 Fe.Their results have challenged the theory of enhanced iron absorption. Lately the same authors (Bannerman, Callender, Hardisty and Sephton Smith, 1964) have extended their investigations to homozygous thalassaemic children as well as heterozygous adults. Although there was wide scatter in iron absorption in their subjects, the mean absorption in both groups did not differ from the normal control receiving the same form of iron. The present study is intended further to investigate iron absorption in thalassaemia. MATERIAL AND METHODSThe clinical material studied consisted of five unrelated families. The diagnosis of thalassaemia was based on the clinical picture, the characteristic morphology of the peripheral blood and the biochemical findings. In each instance at least one member of the family has suffered or was suffering from the honiozygous form of the disease while others were classified as thalassaemia minor or were found to be normal. In all these subjects routine haematological investigations (haemoglobin, haematocrit, red cell morphology, red cell osmotic fragility, reticulocyte count, etc.) as well as biochemical studies (serum iron and ironbinding capacity, determination of foetal haemoglobin and the A2 fraction, etc.) were performed according to standard methods. Diagnosis of the maj...

Five cases of primary intestinal lymphoma are described. The main clinical features included abdominal pain, diarrhea, and marked weight loss, together with radiologic and some laboratory findings suggestive of malabsorption. Laparatomy perfomed in four cases revealed dilatation of the small intestine, with mesenteric node enlargement. In these four instances there was a definite histologic evidence of malignant lymphoma, either initially or later in the course of the disease. A characteristic feature here was the diffuse infiltration of the intestinal mucosa with plasma cells, which in the deeper layers became progressively atypical and were mixed with histiocytes and giant cells. A similar infiltrate was seen in the mesenteric nodes. Immunoelectrophoresis showed the pattern of IgA heavy chain disease. It is suggested that the latter is a variant of primary intestinal lymphoma and not a separate disease entity.

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