Abstract
Hemophagocytic syndrome (HPS) is an unusual but severe illness associated with a variety of infections, as well as genetic, malignant tumors, and autoimmune diseases. We report an 11-year-old girl with systemic lupus erythematosus and nephritis who developed HPS associated with Epstein-Barr virus reactivation. In our patient, the onset of reactive HPS might be related to immunosuppressive treatment during the course of lupus nephritis.
This is a preview of subscription content, log in via an institution to check access.
References
Fisman DN (2000) Hemophagocytic syndromes and infection. Emerg Infect Dis 6:601–608
Sekigawa I, Suzuki J, Nawata M, Ikeda K, Koide M, Iida N Hashimoto H, Oshimi K (2001) Hemophagocytosis in autoimmune disease. Clin Exp Rheumatol 19:333–338
Wong KF, Hui PK, Chan JK, Chan YW, Ha SY (1991) The acute lupus hemophagocytic syndrome. Ann Intern Med 114:387–390
Papo T, Andre MH, Amoura Z, Lortholary O, Tribout B, Guillevin L, Piette JC (1999) The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus. J Rheumatol 26:927–930
Imasuku S, Hibi S, Fujiwara F, Ikushima S, Todo S (1994) Haemophagocytic lymphohistiocytosis, interferon-gamma-nemia and Epstein-Barr virus involvement. Br J Haematol 88:656–658
Lay JD, Tsao CJ, Chen JY, Kadin ME, Su IJ (1997) Upregulation of tumor necrosis factor-alpha gene by Epstein-Barr virus and activation of macrophages in Epstein-Barr virus-infected T cells in the pathogenesis of hemophagocytic syndrome. J Clin Invest 100:1969–1979
Reiner A, Spivak J (1988) Hemophagocytic histiocytosis: a report of 23 new patients and a review of the literature. Medicine (Baltimore) 67:369–388
Janka G, Imashuku S, Elinder G, Schneider M, Henter JI (1998) Infection- and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis. Haematol Oncol North Am 12:435–444
Gill D, Spencer A, Cobcroft R (1994) High-dose gammaglobulin therapy in the reactive haemophagocytic syndrome. Br J Haematol 88:204–206
Seidel MG, Kastner U, Minkov M, Gadner H (2003) IVIG treatment of adenovirus infection-associated macrophage activation syndrome in a two-year-old boy: case report and review of the literature. Pediatr Hematol Oncol 20:445–451
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Isome, M., Suzuki, J., Takahashi, A. et al. Epstein-Barr virus-associated hemophagocytic syndrome in a patient with lupus nephritis. Pediatr Nephrol 20, 226–228 (2005). https://doi.org/10.1007/s00467-004-1704-9
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-004-1704-9