“Mediterranean abdominal lymphoma” or immunoproliferative small intestinal disease.Part II: Pathological aspects

Abstract

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.

Plasma cell infiltration of the small bowel: lack of evidence for a non-secretory form of alpha-heavy chain disease.

“…The IPSID spectrum of disorders includes malignant conditions such as immunoblastic (Mediterranean-type) lymphoma, alpha-chain disease (as-CD) with or without associated lymphoma, and 'benign' conditions characterised by malabsorption with a dense lymphoplasmacytic infiltration of the bowel in patients without lymphoma, and in whom alpha-chains cannot be identified. 2 It has been postulated that diffuse intestinal plasma cell infiltration resulting from possible antigenic stimulation leads in susceptible individuals to the eventual production of as-CD protein;3 and this process may ultimately evolve to lymphoma.4 Although ax-CD protein has been found in the serum in 20-64% of cases of IPSID, 5 6 it has been suggested that the vast majority of cases of IPSID are in fact o-CD. 7 The recent report of a nonsecretory form of a-CD8 has prompted a reevaluation of the prevalence of a-CD in IPSID patients.…”

Bone marrow involvement in a patient with alpha heavy chain disease:Response to tetracycline treatment

“…The immunoglobulin A level is generally not increased. Bone marrow involvement of disease such as is very rare 14. In the current report, we present a case of α - HCD with bone marrow involvement and complete clinical response to tetracycline treatment in a young male.…”

“…It can involve stomach, colon, liver, peritoneum, periaortic and cervical lymph nodes 15. The involvement of bone marrow and increase of immunoglobulin A level in α-HCD is very rare 14. The histological findings of α-HCD have been classified into three distinct stages: Stages A includes mature plasmacytic or lymphoplasmacytic infiltration of mucosal lamina propria and mesenteric lymph nodes.…”

“…Because of the frequent presence of small intestinal bacterial overgrowth and progression to malabsorbtion, the recommended treatment for stage A has been broad spectrum antibiotics with or without corticosteroids 14–19. The average complete remission rate treated with antibiotics is reported to be around 30–70% Treatment duration, was six months, minimally 4.…”

Primary lymphomas of the small intestine: East‐west contrast

“…In the early stage, which Khojasteh et a1 [14] termed a "prelymphomatous phase," the mucosa of long segments of the upper small intestine shows a dense, diffuse infiltration (Fig. 1B) by plasma cells and small lymphocytes [8,10,11,14,56]. The infiltrate separates the crypts and effaces the villous pattern partially or completely.…”