Pathological study of alpha-chain disease, with special emphasis on evolution.
The pathology of six cases of alpha-chain disease (alpha-CD), four of which were followed until complete remission or death, was studied by histologic, immunofluorescence and ultrastructural techniques. The lesions could be classified in three histopographical stages. The late stage C is an immunoblastic sarcoma probably deriving from the same clone as the initial plasmacytic stage A, stage B being a transitional one between A and C. The asynchronism of the lesions in different organs in the same patient requires a laparotomy for an accurate staging which determines the prognosis and the treatment. Complete and prolonged remissions have been observed at stage A only, sometimes with oral antibiotic treatment alone. At all stages, alpha-CD and the "Mediterranean lymphoma" share identical aetiological, clinical and pathological features. Accurate immunological studies will determine the precise-frequency of alpha-CD protein synthesis in the latter syndrome.
D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D002999:Clone Cells / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006362:Heavy Chain Disease / Q000097:blood / Q000209:etiology / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007143:Immunoglobulin Heavy Chains; D007137:Immunoglobulin alpha-Chains; D007413:Intestinal Mucosa / Q000473:pathology; D007414:Intestinal Neoplasms / Q000473:pathology; D007421:Intestine, Small / Q000473:pathology; D008099:Liver / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008223:Lymphoma / Q000473:pathology; D008297:Male; D008643:Mesentery; D008875:Middle Aged; D010950:Plasma Cells / Q000473:pathology; D011379:Prognosis; D012007:Rectum / Q000473:pathology; D013154:Spleen / Q000473:pathology; D013270:Stomach / Q000473:pathology
D000900:Anti-Bacterial Agents; D007143:Immunoglobulin Heavy Chains; D007137:Immunoglobulin alpha-Chains