Primary Epstein-Barr virus infection with clonal T-cell lymphoproliferation

A case of fatal Epstein-Barr virus infection in a previously healthy girl who was first found to have severe infectious mononucleosis with spontaneous recovery is reported. Because an abnormal immune response to the virus persisted, the disease relapsed, manifesting in cutaneous and pulmonary lesion...

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Bibliographic Details
Published in:	American journal of clinical pathology Vol. 98; no. 3; p. 324
Main Authors:	Gaillard, F, Mechinaud-Lacroix, F, Papin, S, Moreau, A, Mollat, C, Fiche, M, Peltier, S, De Faucal, P J, Rousselet, M C, Praloran, V
Format:	Journal Article
Language:	English
Published:	England 01-09-1992
Subjects:	Adolescent Blotting, Southern Female Gene Rearrangement, T-Lymphocyte - genetics Herpesvirus 4, Human - isolation & purification Humans Immunohistochemistry Infectious Mononucleosis - complications Infectious Mononucleosis - genetics Infectious Mononucleosis - pathology Lymphoproliferative Disorders - genetics Lymphoproliferative Disorders - microbiology Lymphoproliferative Disorders - pathology Nucleic Acid Hybridization T-Lymphocytes
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Summary:	A case of fatal Epstein-Barr virus infection in a previously healthy girl who was first found to have severe infectious mononucleosis with spontaneous recovery is reported. Because an abnormal immune response to the virus persisted, the disease relapsed, manifesting in cutaneous and pulmonary lesions associated with hemophagocytic syndrome responsible for death. Pathologic findings were characterized by polymorphous atypical lymphoid infiltrate, prominent necrosis, and histiocytic hyperplasia. Lymphoid cells displayed CD8 phenotype and clonal T-cell receptor gene rearrangement. Viral genome was detected in lesions by Southern blot and located in nuclei of lymphoid cells by in situ hybridization. Pathologic findings suggested fatal infectious mononucleosis; however, phenotype and genotype favored a malignant diagnosis. Clonality was demonstrated to have arisen during primary infection. Virologic examination indicated that Epstein-Barr virus was a causative agent. Such a process belongs to the recently recognized spectrum of Epstein-Barr virus-related T-cell lymphoproliferative disorders that might overlap fatal infectious mononucleosis in patients who are especially vulnerable to the virus.
ISSN:	0002-9173
DOI:	10.1093/ajcp/98.3.324