Pathological study of alpha-chain disease, with special emphasis on evolution

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Abstract
The pathology of six cases of alpha-chain disease (α-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 histotopographical 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, α-CD and the “Mediterranean lymphoma” share identical aetiological, clinical and pathological features. Accurate immunological studies will determine the precise frequency of α-CD protein synthesis in the latter syndrome.