European Journal of Pediatrics

, Volume 150, Issue 1, pp 48–53 | Cite as

Epstein-Barr virus infection rapidly progressing to monoclonal lymphoproliferative disease in a child with selective immunodeficiency

  • V. Schuster
  • H. W. Kreth
  • H. K. Müller-Hermelink
  • H. -I. Huppertz
  • A. C. Feller
  • D. Neumann-Haefelin
  • H. Wiegand
  • N. Müller-Lantzsch
Infectious Diseases

Abstract

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 β-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 immunodeficiency had led to the rapid development of EBV-associated monoclonal lymphoproliferation.

Key words

Epstein-Barr virus Lymphoproliferative syndrome Inherited immunodeficiency 

Abbreviations

B-LCL

EBV-transformed lymphoblastoid B-cell line

c-myc

member of the myc oncogene family, related to the viral myc oncogen isolated from Avian Myelocytomatosis Virus

EA

early antigen

EBV

Epstein-Barr virus

EBNA

Epstein-Barr nuclear antigen

LNC

lymph node cells

OAS

2′–5′-oligoadenylate synthetase

PBMC

peripheral blood mononuclear cells

TCL

T cell line

TcR

T cell receptor

VCA

viral capsid antigen

XLP

X-linked lymphoproliferative syndrome

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Copyright information

© Springer-Verlag 1990

Authors and Affiliations

  • V. Schuster
    • 1
  • H. W. Kreth
    • 1
  • H. K. Müller-Hermelink
    • 2
  • H. -I. Huppertz
    • 1
  • A. C. Feller
    • 2
  • D. Neumann-Haefelin
    • 3
  • H. Wiegand
    • 3
  • N. Müller-Lantzsch
    • 4
  1. 1.Department of PaediatricsUniversity of WürzburgWürzburgFederal Republic of Germany
  2. 2.Department of PathologyUniversity of WürzburgWürzburgFederal Republic of Germany
  3. 3.Department of VirologyUniversity of FreiburgFreiburgFederal Republic of Germany
  4. 4.Department of VirologyUniversity of HomburgHomburgFederal Republic of Germany

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