The evolution of alpha heavy chain disease

J. Guardia, J. Rubiés-Prat, M. T. Gallart, A. Moragas, J. M. Martínez-Vázquez, R. Bacardí, J. Vilaseca

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Abstract

Two patients with alpha heavy chain disease are described. In the first patient, treatment with cyclophosphamide, prednisone and doxycycline was associated with a 28 month long remission and the disappearance of the paraprotein and lymphoplasmocytic infiltration of the intestine. Shortly afterwards, a retroperitoneal immunoblastic lymphoma was found associated with an immunoglobulin G kappa paraproteinemia, and gamma heavy and kappa light chains in the urine; the intestinal biopsy specimen was normal. In the other patient, the alpha chain only appeared two years after the malabsorption syndrome. The fact that in the first, apparently cured patient, a tumor of different anatomic site and secretory capacity appeared, suggests the existence of a B cell neoplasia of different clone from that which gave rise to the original disease. In the second patient, it is probable that only the increase in the mass of neoplastic cells led to the detection of the protein abnormality, or alternatively the antigenic oncogenic stimulus led to the abnormal secretion only after two years.
Original languageEnglish
Pages (from-to)596-602
JournalAmerican Journal of Medicine
Volume60
Issue number4
DOIs
Publication statusPublished - 1 Jan 1976

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    Guardia, J., Rubiés-Prat, J., Gallart, M. T., Moragas, A., Martínez-Vázquez, J. M., Bacardí, R., & Vilaseca, J. (1976). The evolution of alpha heavy chain disease. American Journal of Medicine, 60(4), 596-602. https://doi.org/10.1016/0002-9343(76)90723-3