No 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.

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.

A B S T R A C T The peripheral blood lymphocytes of nine patients with hyper immunoglobulin (Ig)M immunodeficiency were studied in an attempt to define the cellular basis of this disorder. B cells were normal in number but qualitatively abnormal in all patients.Approximately one-half of the B cell consisted of small lymphocytes (7-9 ,um

The rare strains of staphylococci which lack either free coagulase or bound ceagulase (the clumping factor responsible for agglutination and compact colony formation in fibrinogen) , but not both these properties, have been considered to be od doubtful pathogenicity( 1). However, Johanovsky ( 2 ) suggested that the clumping property rather than free colagulase may allow survival ol f staphylococci within phagocytes. Hunt and Moses (3) reported that only one of 2 serologically distinct variants of the Smith strain of Staphylococcus uu~t'us was virulent for mice although both were coagulase positive. The mouse virulent variant formed diffuse colonies iin plasma soft agar, whereas the cololnies of the avirulent variant were compact in this mediurn. Alami and Kelly( 4) concluded that the coimpacting of staphylococcal colonies in fibrinogen-containing soft agar is an expression of the clumping factor and need not be due exclusively to antibody action. The present study was initiated to compare free coagulase anid bound coagulase as virulence factors and to determine whether route of injection affected the results in mice.Materials and nzefhods. Twelve strains representing typical coagulase positive and cmgulase negative staphylococci as well as variants which lack either free or bound coagulase (hereafter referred to as coagulase and clumping factor, respectively) or both these properties were selected for study. The compact colony (S) and diffuse colony (Sv) forming variants of the Smith strain were kindly supplied by Dr. G. A. Hunt. Others were isolated locally from hospital personnel and out-patients or were variants of these strains. Coagulase and clumping factor activities were determined by the soft agar technic(4) as well as by conventional methods.