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Alpha heavy chain disease in a child

Seligmann in 1968.1 The disorder is characterized by plasma cell infiltration of the intestine with production of incomplete immunoglobulin A heavy chains in the absence of light chains. It appears to have a premalignant phase (immunoproliferative small intestinal disease; IPSID), which is followed in most cases by the development of an immunoblastic lymphoma of the small intestine.2 In contrast to other HCD, α-HCD has a predilection for younger age groups, including children.3 In the present report, we present a 12-year-old boy with alpha chain disease and evidence of malignant lymphoma 1 year after the initial diagnosis.