The Digestive Form of α-Chain Disease

  • Maxime Seligmann
  • Jean-Claude Rambaud
Part of the Sloan-Kettering Institute Cancer Series book series (SKICS)


Alpha-chain disease (α-CD) is a proliferative disorder of B-lymphoid cells involving primarily the IgA secretory system, in which plasma cells produce a presumably homogeneous population of immunoglobulin molecules consisting of incomplete a chains devoid of light chains. Since the first description of this new immunoglobulin abnormality (Seligmann et al., 1968) in a young Syrian patient affected with malabsorption and diffuse plasmacytic infiltration of the small intestine (Rambaud et al., 1968), more than 100 cases have been recognized to our knowledge. Alpha-chain disease is thus the most frequent of the heavy-chain diseases. In three of these patients a-CD was apparently confined to the respiratory tract (Stoop et al., 1971; Faux et al., 1973; Florin-Christensen et al., 1974). All the other patients were affected with the digestive form of a-CD, which is mainly localized in the small intestine and the mesenteric lymph nodes.


Plasma Cell Light Chain Chronic Lymphocytic Leukemia Mesenteric Lymph Node Plasma Cell Infiltration 
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Copyright information

© Springer Science+Business Media New York 1978

Authors and Affiliations

  • Maxime Seligmann
    • 1
  • Jean-Claude Rambaud
    • 2
  1. 1.Laboratory of Immunochemistry and Immunopathology (INSERM U 108), Research Institute on Blood DiseasesHôpital Saint-LouisParis 10France
  2. 2.Department of Gastroenterology and Research Unit on Physiopathology of Digestion (INSERM U 54)Hôpital Saint-LazareParis 10France

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