Lymphoproliferative disease associated with immune deficiency in children.

Abstract

Lymphoproliferations in patients with immune deficiency form a challenge for the pathologist. The histologic features often are not specific and might even be deceptive. The demonstration of the presence of the Epstein-Barr virus is important, preferably by the most sensitive technique, Epstein-Barr virus-encoded small RNA in situ hybridization. In more aggressive cases, a lymphoma can be diagnosed and classified according to the standard criteria, but in contrast with lymphomas that arise in immunocompetent patients, not all cases are monoclonal. Although light-chain restriction often can be demonstrated, even on paraffin, thanks to the plasmacytic differentiation that often is present, the most reliable technique is molecular demonstration of a clonal antigen receptor rearrangement. The workshop showed in addition that many unusual cases arise in immunodeficient patients.