Radiological and histological findings in six patients with alpha-chain disease

Detailed radiological and histological features of alpha-chain disease are presented against a background of the clinical and biochemical findings. The separation of this syndrome from other varieties of "Mediterranean" lymphoma is emphasized. The radiologist should in some instances be able to suggest the diagnosis in his differential diagnosis of abnormalities seen on examination of the small bowel. The histopathologist should be able to provide a diagnosis from examination of a peroral jejunal biopsy. One patient with this relatively uncommon disease had evidence of a hypertrophic osteoarthropathy. Two of the six patients had a transient episode of acute abdominal distension which resolved on conservative management.