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  • Title: Pathological study of alpha-chain disease, with special emphasis on evolution.
    Author: Galian A, Lecestre MJ, Scotto J, Bognel C, Matuchansky C, Rambaud JC.
    Journal: Cancer; 1977 May; 39(5):2081-101. PubMed ID: 404026.
    Abstract:
    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.
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