Epstein-Barr virus infection rapidly progressing to monoclonal lymphoproliferative disease in a child with selective immunodeficiency.
We report on a 30-month-old previously healthy Turkish boy who presented with fever, hepatosplenomegaly and generalized lymphadenopathy. He died 4 months after admission in spite of treatment with steroids, acycloguanosine and cyclophosphamide. Epstein-Barr virus (EBV) DNA was detected in the patient's bone marrow and in a lymph node biopsy. Cells from the lymph node biopsy showed monoclonal rearrangements of immunoglobulin heavy chain genes but no rearrangements of T-cell receptor beta-chain genes or immunoglobulin kappa chain genes. Serological data indicated chronic active EBV infection. There was a slight increase of CD8 positive cells in peripheral blood and a normal response to T-cell mitogens. However, T-cell lines established with interleukin 2 from lymph node biopsy completely failed to kill autologous EBV-transformed B-cells and K 562 target cells. Moreover, in regression tests the patient's peripheral blood mononuclear cells completely failed to limit outgrowth of autologous EBV infected B-cells. We conclude that the patient's selective immuno-deficiency had led to the rapid development of EBV-associated monoclonal lymphoproliferation.
Schuster
V
V
Department of Paediatrics, University of Würzburg, Federal Republic of Germany.
Kreth
H W
HW
Müller-Hermelink
H K
HK
Huppertz
H I
HI
Feller
A C
AC
Neumann-Haefelin
D
D
Wiegand
H
H
Müller-Lantzsch
N
N
D000914:Antibodies, Viral / Q000032:analysis; D002675:Child, Preschool; D016259:Genes, myc; D008040:Genetic Linkage; D006566:Herpesviridae Infections / Q000209:etiology* / Q000276:immunology; D004854:Herpesvirus 4, Human; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000235:genetics / Q000276:immunology; D016131:Lymphocyte Subsets; D008232:Lymphoproliferative Disorders / Q000209:etiology*; D008297:Male; D014960:X Chromosome
D000914:Antibodies, Viral