Abstract
α-Chain disease (α-CD) is defined as a B-lymphoid cell proliferation involving first and foremost the IgA secretory system and producing a presumably homogeneous population of immunoglobulin molecules consisting of incomplete α-chains and devoid of light chains. It is the most frequent of the heavy chain diseases and, since its initial description in 1968 [15, 26], more than 120 cases have been recognized to our knowledge. All these patients, except three who showed an involvement of the respiratory tract, were affected with the digestive form of α-CD which is mainly located in the small intestine and the mesenteric lymph nodes.
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