T-cell lymphoma and the virus-associated hemophagocytic syndrome

• Published in: American journal of clinical pathology Vol. 97; no. 2; pp. 189 - 194
• Main Authors: CRAIG, F. E, CLARE, C. N, SKLAR, J. L, BANKS, P. M
• Format: Journal Article
• Language: English
• Published: Chicago, IL American Society of Clinical Pathologists 01.02.1992
• Subjects: Biological and medical sciences; Blotting, Southern; DNA; Epstein-Barr virus; Female; Hematologic and hematopoietic diseases; Herpesvirus 4, Human; Histiocytosis, Non-Langerhans-Cell - complications; Histiocytosis, Non-Langerhans-Cell - pathology; Humans; Immunohistochemistry - methods; Infant; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymph Nodes - pathology; Lymphoma, T-Cell - complications; Lymphoma, T-Cell - pathology; Medical sciences; Nucleic Acid Hybridization; Staining and Labeling; Tumor Virus Infections - complications; Gammaherpesvirinae; Virus; Human; Herpesviridae; T-Lymphocyte; Malignant hemopathy; Epstein Barr virus; Lymphoma
• ISSN: 0002-9173; 1943-7722
• DOI: 10.1093/ajcp/97.2.189

A 20-month-old girl had a disorder that by both clinical and histologic criteria resembled the virus-associated hemophagocytic syndrome in the setting of Epstein-Barr virus infection. Subsequent investigation revealed histologic evidence of disseminated T-cell lymphoma. DNA hybridization studies displayed a monoclonal T-cell receptor beta chain rearrangement, in the absence of clonal immunoglobulin gene rearrangement, and a single band in the analysis for the fused termini of the Epstein-Barr virus genome. These results suggest the presence of a monoclonal population of T lymphocytes infected with Epstein-Barr virus. The diagnosis of lymphoma was confirmed at autopsy. The authors discuss the association of Epstein-Barr virus infection with the development of T-cell lymphoma and propose that the previous reports of virus-associated hemophagocytic syndrome include cases of unrecognized T-cell lymphoma.