Abstract
A review of alpha-heavy chain disease (AHCD) emphasizing its histopathology and associated lesions is presented. Unusual clinicopathologic presentations and modern concepts regarding histogenesis of AHCD in the light of recent immunohistochemical findings are discussed. Increasingly reported cases of Burkitt's (or Burkitt-type) lymphoma involving distal small intestine or the ileocecal region in children are noted.
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