title: [Clinico-pathological study of a case of alpha chain disease].
abstract: A case of alpha-chain disease is presented. Diagnosis was clinched by the discovery of typical heavy alpha-chains on analysis of serum proteins. The clinical picture included non-gluten-dependent semi-coeliac malabsorption, Hippocratic fingers, flattened villi, plasma cell infiltration of the jejunal mucosa, alpha- and beta-globulin dysprotidaemia, and abdominal masses; these signs are indicative of, but not conclusive evidence of alpha-chain disease. The presence of Coccidioides organules in the mucosa and glandular tuberculosis rises aetiopathogenetic questions that suggest that due attention be given to microbiological examination. Rapid progression to neoplasia following immunodepressive management underscores the risks associated with the administration of immunodepressors in patients with immunitary deficiencies. It is still not clear whether alpha-chain disease should be regarded as a self-perpetuating immunoproliferative disease, or as a form neoplasia ab initio, with a protracted, though of inevitably fatal, course.
mesh_terms: D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D006362:Heavy Chain Disease / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007143:Immunoglobulin Heavy Chains; D007137:Immunoglobulin alpha-Chains; D007166:Immunosuppressive Agents / Q000009:adverse effects* / Q000627:therapeutic use; D007421:Intestine, Small / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008223:Lymphoma / Q000276:immunology; D008297:Male
chemical_list: D007143:Immunoglobulin Heavy Chains; D007137:Immunoglobulin alpha-Chains; D007166:Immunosuppressive Agents
vernacular_title: Studio clinico patologico di un caso di alpha chain disease