melphalan and Protein-Losing-Enteropathies

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.

Topics: Adrenal Cortex Hormones; Adult; Biopsy; Heavy Chain Disease; Humans; Immunoglobulin alpha-Chains; Immunoglobulin Heavy Chains; Intestine, Small; Malabsorption Syndromes; Male; Melphalan; Plasma Cells; Protein-Losing Enteropathies; Remission, Spontaneous