Abstract
Epstein-Barr virus (EBV) is known to cause infectious mononucleosis (1), a usually benign and self-limiting B-cell lymphoproliferative disorder. In addition, the viral infection is also associated with several forms of histiocytic (2) and lymphoproliferative syndromes (3), mainly in patients with inherited or acquired immunodeficiencies (4.5.6.7). Many patients reveal various subtle T-cell and B-cell defects. The X-linked lymphoproliferative syndrome (XLP), also known as Duncan disease (8), is characterized by the inability of affected individuals to mount an adequate immune response to EBV (9). The genetic locus of ILP could be located in the Xq25-q26 vicinity in some affected families(10.11). After primary EBV infection affected family members present with severe or fatal IM, acquired hypogammglobulinemia. aplastic anemia, B-cell lymphoproliferative disease. and virus-associated hemophagocytic syndrome (VAHS)(12.13).
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Similar content being viewed by others
References
C.E. Jr Rapp, J. F. Hewetson, Am.J. Dis. Child 132, 78–86 (1978)
K. McClain, R. Gehrz. et al. Am. J Pediatr. Hematol. Oncol. 10, 195–205, (1988)
D.W. Hanto, G. Frizzera, et al,N. Engl. J. Med. 306, 913–918, (1982)
W. Andiman, K. Eastman, et al.,Lancet, 1390–1393, (1985)
M. Ho, G. Miller. et al, J. Infect. Dis. 152, 876–886
J.H. Kersey, R.S. Shapiro, et al, Pediatr. Infect Dis. 7(5 Suppl,), 10–12, (1988)
W.T. Shearer, J. Ritz, et al, N. Enpl. J. Med. 312, 1151–1159, (1985)
D.T. Purtilo, C. Cassel, et al, Lancet, 935–941, (1975)
D.T. Purtilo, K. Sakamoto, et al, Cancer Res. 41, 4226–4235, (1981)
J.C. Skare, A. Milunsky, et al, Froc. Natl. Acad. Sci. U.S.A. 84, 2015–2019, (1987)
J.C. Skare, H.L. Grierson. et al. Hum. Genet. 82. 354–358, (1989)
H. Grierson, D.T. Purtilo. Ann. Intern. Med. 106, 538–545, (1987)
J.L. Sullivan, B.A. Woda. et al, Blood 65. 1097–1104,(1985)
J. Joncas, F. Monczak. et al, J Med. Virol. 28, 110–117, (1989)
D. Hellmann, M.J. Cowan. et al, J. Pediatr. 103, 585–588, (1983)
H.M. Lederman, R. Yolken. et al, Pediatr. Infect Dis. 2, 388–390, (1983)
R.E. Scully, E.J. Mark, et al, N. Engl. J. Med. 323–984, (1990)
Author information
Authors and Affiliations
Editor information
Rights and permissions
Copyright information
© 1991 Springer Science+Business Media New York
About this chapter
Cite this chapter
Schuster, V., Kreth, H.V. (1991). Epstein-Barr Virus Associated Histio-Lymphoproliferative Syndromes in Two European Families. In: Ablashi, D.V., Huang, A.T., Pagano, J.S., Pearson, G.R., Yang, C.S., Ablashi, K.L. (eds) Epstein-Barr Virus and Human Disease · 1990. Experimental Biology and Medicine, vol 24. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-4612-0405-3_20
Download citation
DOI: https://doi.org/10.1007/978-1-4612-0405-3_20
Publisher Name: Humana Press, Totowa, NJ
Print ISBN: 978-1-4612-6747-8
Online ISBN: 978-1-4612-0405-3
eBook Packages: Springer Book Archive