title: Malabsorption associated with a high-grade-malignant non-Hodgkin's lymphoma, alpha-heavy-chain disease and immunoproliferative small intestinal disease.
abstract: We report on a 28 year old Turkish woman, who was admitted to our hospital with the symptoms of malabsorption and protein-loosing enteropathy. Histologically, on duodenal biopsy, a lymphoplasmacellular infiltration of the submucosa with partial to subtotal atrophy of the villi was found. An immunoproliferative small intestinal disease (IPSID) was diagnosed. A short remission whilst on a glutenfree diet and tetracycline therapy, was followed by a laparatomy because of ileus in the small intestine. A high-grade-malignant Non-Hodgkin's Lymphome of B-cell type with intracellular production of alpha-Heavy-Chains (AHCD) was diagnosed histologically. Following chemotherapy with CEOP-IMVP-Dexa (Cyclophosphamide, Epidoxorubicin, Vincristine, Prednisolone, Ifosfamide, VP-16, Dexamethason, Methotrexat) the patient is still in complete remission three years after starting the therapy. We discuss here a case of AHCD in IPSID, the differential diagnosis of protein losing enteropathy and malabsorption, and we also present conservative (diet, medical treatment) and operative therapies.
authors.affiliation: II. Department of Internal Medicine, General Hospital Linz, Austria.
authors.forename: R
authors.initials: R
authors.lastname: Hubmann
authors.forename: W
authors.initials: W
authors.lastname: Kaiser
authors.forename: T
authors.initials: T
authors.lastname: Radaszkiewicz
authors.forename: M
authors.initials: M
authors.lastname: Fridrik
authors.forename: I
authors.initials: I
authors.lastname: Zazgornik
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy; D004386:Duodenum / Q000473:pathology; D005260:Female; D006362:Heavy Chain Disease / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007161:Immunoproliferative Small Intestinal Disease / Q000473:pathology* / Q000628:therapy; D007413:Intestinal Mucosa / Q000473:pathology; D007414:Intestinal Neoplasms / Q000473:pathology* / Q000628:therapy; D007415:Intestinal Obstruction / Q000473:pathology / Q000628:therapy; D016393:Lymphoma, B-Cell / Q000473:pathology* / Q000628:therapy; D008228:Lymphoma, Non-Hodgkin / Q000473:pathology* / Q000628:therapy; D008286:Malabsorption Syndromes / Q000473:pathology* / Q000628:therapy; D012074:Remission Induction