Immunoproliferative Small Intestinal Disease with 40-years of follow-up: A case of cured α-chain disease with recurrent lymphoplasmacytic tumors of the small intestine (MALT lymphomas)

Authors O.N. Manousos, J. Economidou, J.K. Triantafillidis, A. Karameris, N. Kafasi.


Background: Immunoproliferative small intestinal disease (IPSID) represents a spectrum of clinicopathological entities including alpha-chain disease (α-CD) and other types of lymphoplasmacytic proliferations of the lamina propria of the small intestine, presenting with severe malabsorption. The disease may progress from a low grade MALT B-cell lymphoma to a high grade immunoblastic lymhoma.We present here a case of α-CD with an unusual evolution and long survival. Case - report: A 27 years old Greek man developed in 1967 severe diarrhea, abdominal pain and loss of weight.The diarrhea responded to treatment with antibiotics temporarily.His symptoms fluctuated and in 1971 the diagnosis of alpha chain disease was made.Treatment with cyclophosphamide and antibiotics until June of 1972 was followed by complete clinical recovery, return of histological appearances of the small intestine to normal and disappearance of the alpha chain proteins from the serum. He was symptom free without additional treatment until January of 1974 when he developed an ileocaecal tumor which was surgically resected.Histological examination concluded that it was a plasmacytoma consisting of 68% α cells and 15% λ cells. The patient had a course of radiotherapy and was put on maintenance treatment with cyclophosphamide. In 1977 he developed another tumour of the terminal ileum which was also resected. Histologically it was characterized as B-cell immunocytoma.He was treated with cyclophosphamide for 12 months. In December 1978 he developed again symptoms of abdominal neoplasm. At operation an inoperable tumour involving the small intestine and the mesenteric lymph nodes was found.Histological examination characterized it as a B-cell immunocytoma.He was treated with a monthly scheme of COP for two years (1979-1981) with success. Since then he is in perfect physical condition. Conclusion: According to recent studies this exceptional case, in retrospect, may be characterized as a cured case of α-CD with reccurent low grade MALT lymphomas of the small intestine showing extensive plasmacytic differentiation. The patient has been in perfect health for the past 26 years. Recent thorough investigation including immunological and molecular studies showed no evidence of alpha-chain disease or lymphoma.
Case Reports