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

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. Copyright © 1977 American Cancer Society