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Chapter 54 Infectious Mononucleosis and Other Epstein-Barr Virus–Associated Diseases 759

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only received chemotherapy. A pivotal, randomized clinical study lymphohistiocytosis undergoing allogeneic hematopoietic cell transplan-
to validate these findings is in progress. tation. Blood 116:5824–5831, 2010.
13. Imashuku S: Treatment of Epstein-Barr virus-related hemophagocytic
lymphohistiocytosis (EBV-HLH); update 2010. J Pediatr Hematol Oncol
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14. Chellapandian D, Das R, Zelley K, et al: Treatment of Epstein Barr
Since its discovery in 1964, EBV has been linked to a heterogeneous virus-induced haemophagocytic lymphohistiocytosis with rituximab-
group of diseases. EBV was the first human virus implicated in containing chemo-immunotherapeutic regimens. Br J Haematol
oncogenesis, and the biology of the virus has been studied extensively 162:376–382, 2013.
on a cellular and molecular level. Over the last two decades immu- 15. Filipovich AH, Zhang K, Snow AL, et al: X-linked lymphoproliferative
notherapeutic approaches targeting EBV have been developed for syndromes: brothers or distant cousins? Blood 116:3398–3408, 2010.
EBV-associated malignancies and later phase clinical studies are cur- 16. Cohen JI, Kimura H, Nakamura S, et al: Epstein-Barr virus-associated
rently in progress. Although adoptive transfer of T cells so far has lymphoproliferative disease in non-immunocompromised hosts: a status
been more successful than vaccines, novel, more potent vaccines have report and summary of an international meeting, 8–9 September 2008.
been developed that await testing in humans. Combinatorial thera- Ann Oncol 20:1472–1482, 2009.
pies, for example combining EBV-targeted immunotherapy with 17. Ohshima K, Kimura H, Yoshino T, et al: Proposed categorization of
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ated in clinical studies and hold the promise to improve outcomes. liferative disorder (LPD) in children and young adults: overlap with
In the next 5 years, rationally designed small molecule inhibitors will chronic active EBV infection and infantile fulminant EBV T-LPD. Pathol
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exome sequencing will continue to identify genes that are associated topoietic SCT with reduced-intensity conditioning for the treatment of
with life-threatening complications of EBV infections, providing chronic active EBV infection. Bone Marrow Transplant 46:77–83, 2011.
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