title: [Alpha-chain disease presenting as malabsorption syndrome with exudative enteropathy (author's transl)].
abstract: A 30-year-old Turk was admitted with signs of exudative enteropathy together with malabsorption. There was no improvement on a gluten-free diet. Immunological investigations demonstrated atypical IgA-immunoglobulin in the serum which did not precipitate with antisera against L-chains. Peroral ileal biopsy and surgical biopsy material showed a diffuse proliferation of plasma cells in an altered ileal mucosa and in the mesenteric lymph nodes. Skeletal X-rays showed no osteolysis and the bone marrow showed no evidence for multiple myeloma. Treatment with melphalan and steroids resulted in a three year remission. In the terminal stage an intra-abdominal malignant lymphoma developed.
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D001706:Biopsy; D006362:Heavy Chain Disease / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007143:Immunoglobulin Heavy Chains; D007137:Immunoglobulin alpha-Chains; D007421:Intestine, Small / Q000473:pathology; D008286:Malabsorption Syndromes / Q000209:etiology; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D010950:Plasma Cells / Q000473:pathology; D011504:Protein-Losing Enteropathies / Q000209:etiology* / Q000473:pathology; D012075:Remission, Spontaneous
chemical_list: D000305:Adrenal Cortex Hormones; D007143:Immunoglobulin Heavy Chains; D007137:Immunoglobulin alpha-Chains; D008558:Melphalan
vernacular_title: Alpha-Ketten-Krankheit als Malabsorptionssyndrom mit exsudativer Enteropathie