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The Digestive Form of α-Chain Disease

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

Abstract

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.

Keywords

Plasma Cell Light Chain Chronic Lymphocytic Leukemia Mesenteric Lymph Node Plasma Cell Infiltration 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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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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