Middle East lymphoma and α-chain disease

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Abstract
An immunoperoxidase study of the small intestinal mucosa of 3 patients with .alpha.-chain disease showed heavy infiltration of the mucosa by plasma cells containing .alpha.-heavy chain and J-chain but no light chains. An additional band-like and nodular mucosal infiltrate was also present and consisted of cells showing no evidence of cytoplasmic immunoglobulin synthesis. The cells comprising this infiltrate invaded and destroyed intestinal crypts, and immunoperoxidase staining showed them to be sharply distinct from the .alpha.-chain-containing plasma cells. In 2 cases of Middle East lymphoma, immunohistochemistry revealed a normal plasma cell population in the lamina propria of the small intestine. Alpha-chain disease apparently diagnosed in routine paraffin sections which should permit clarification of its true incidence in Middle East lymphoma. The demonstration of a sharp distinction between the 2 types of mucosal infiltrate in .alpha.-chain disease contrasts with previous immunofluoresence results, and enables more ready identification of mucosal changes that may be important in the management of the disease.