Lymphoproliferative disease associated with immune deficiency in children.
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.
van Krieken
Joannes H
JH
Department of Pathology, University Medical Center Nijmegen, The Netherlands.
D002648:Child; D019674:Genes, T-Cell Receptor beta; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D007108:Immune Tolerance; D007153:Immunologic Deficiency Syndromes / Q000150:complications; D008232:Lymphoproliferative Disorders / Q000235:genetics / Q000276:immunology* / Q000473:pathology / Q000821:virology; D014180:Transplantation / Q000009:adverse effects