Infectious Triggers of Cytokine Storm Syndromes: Herpes Virus Family (Non-EBV)



The herpesviruses are the most common infectious agents associated with both primary and secondary cytokine storm syndromes (CSS). While Epstein–Barr Virus (EBV) is most frequently reported in association with CSS, cytomegalovirus (CMV) and many other herpesviruses (e.g., herpes simplex virus, varicella zoster virus, and human herpesviruses 6 and 8) are clearly associated with CSS in children and adults. Immunocompromised hosts, whether due to primary immunodeficiency or secondary immune compromise (e.g., solid organ or stem cell transplantation), appear to be at particularly increased risk of herpesvirus-associated CSS. In this chapter, the association of the non-EBV herpesviruses with CSS will be discussed, including predisposing factors and treatment considerations.


  1. 1.
    Risdall, R. J., McKenna, R. W., Nesbit, M. E., Krivit, W., Balfour Jr., H. H., Simmons, R. L., et al. (1979). Virus-associated hemophagocytic syndrome: A benign histiocytic proliferation distinct from malignant histiocytosis. Cancer, 44, 993–1002.PubMedPubMedCentralCrossRefGoogle Scholar
  2. 2.
    Behrens, E. M., Canna, S. W., Slade, K., Rao, S., Kreiger, P. A., Paessler, M., et al. (2011). Repeated TLR9 stimulation results in macrophage activation syndrome-like disease in mice. The Journal of Clinical Investigation, 121, 2264–2277.PubMedPubMedCentralCrossRefGoogle Scholar
  3. 3.
    Orange, J. S., Fassett, M. S., Koopman, L. A., Boyson, J. E., & Strominger, J. L. (2002). Viral evasion of natural killer cells. Nature Immunology, 3, 1006–1012.PubMedCrossRefGoogle Scholar
  4. 4.
    Odom, C. I., Gaston, D. C., Markert, J. M., & Cassady, K. A. (2012). Human herpesviridae methods of natural killer cell evasion. Advances in Virology, 2012, 359869.PubMedPubMedCentralCrossRefGoogle Scholar
  5. 5.
    Brisse, E., Wouters, C. H., Andrei, G., & Matthys, P. (2017). How viruses contribute to the pathogenesis of hemophagocytic lymphohistiocytosis. Frontiers in Immunology, 8, 1102.PubMedPubMedCentralCrossRefGoogle Scholar
  6. 6.
    Henter, J. I., Ehrnst, A., Andersson, J., & Elinder, G. (1993). Familial hemophagocytic lymphohistiocytosis and viral infections. Acta Paediatrica, 82, 369–372.PubMedCrossRefGoogle Scholar
  7. 7.
    Henter, J. I., Elinder, G., Soder, O., & Ost, A. (1991). Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatrica Scandinavica, 80, 428–435.PubMedCrossRefGoogle Scholar
  8. 8.
    Otrock, Z. K., & Eby, C. S. (2015). Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. American Journal of Hematology, 90, 220–224.PubMedPubMedCentralCrossRefGoogle Scholar
  9. 9.
    Ramos-Casals, M., Brito-Zeron, P., Lopez-Guillermo, A., Khamashta, M. A., & Bosch, X. (2014). Adult haemophagocytic syndrome. Lancet, 383, 1503–1516.PubMedPubMedCentralCrossRefGoogle Scholar
  10. 10.
    Fisman, D. N. (2000). Hemophagocytic syndromes and infection. Emerging Infectious Diseases, 6, 601–608.PubMedPubMedCentralCrossRefGoogle Scholar
  11. 11.
    Chen, R. L., Lin, K. H., Lin, D. T., Su, I. J., Huang, L. M., Lee, P. I., et al. (1995). Immunomodulation treatment for childhood virus-associated haemophagocytic lymphohistiocytosis. British Journal of Haematology, 89, 282–290.PubMedCrossRefGoogle Scholar
  12. 12.
    Gill, D. S., Spencer, A., & Cobcroft, R. G. (1994). High-dose gamma-globulin therapy in the reactive haemophagocytic syndrome. British Journal of Haematology, 88, 204–206.PubMedCrossRefGoogle Scholar
  13. 13.
    Jordan, M. B., Allen, C. E., Weitzman, S., Filipovich, A. H., & McClain, K. L. (2011). How I treat hemophagocytic lymphohistiocytosis. Blood, 118, 4041–4052.PubMedPubMedCentralCrossRefGoogle Scholar
  14. 14.
    Staras, S. A., Dollard, S. C., Radford, K. W., Flanders, W. D., Pass, R. F., & Cannon, M. J. (2006). Seroprevalence of cytomegalovirus infection in the United States, 1988-1994. Clinical Infectious Diseases, 43, 1143–1151.PubMedCrossRefGoogle Scholar
  15. 15.
    Heininger, A., Jahn, G., Engel, C., Notheisen, T., Unertl, K., & Hamprecht, K. (2001). Human cytomegalovirus infections in nonimmunosuppressed critically ill patients. Critical Care Medicine, 29, 541–547.PubMedCrossRefGoogle Scholar
  16. 16.
    Hummel, M., & Abecassis, M. M. (2002). A model for reactivation of CMV from latency. Journal of Clinical Virology, 25(Suppl 2), S123–S136.PubMedCrossRefGoogle Scholar
  17. 17.
    Amin, M. M., Bialek, S. R., Dollard, S. C., & Wang, C. (2018). Urinary cytomegalovirus shedding in the United States: The National Health and Nutrition Examination Surveys, 1999-2004. Clinical Infectious Diseases, 67(4), 587–592.PubMedPubMedCentralCrossRefGoogle Scholar
  18. 18.
    Cannon, M. J., Stowell, J. D., Clark, R., Dollard, P. R., Johnson, D., Mask, K., et al. (2014). Repeated measures study of weekly and daily cytomegalovirus shedding patterns in saliva and urine of healthy cytomegalovirus-seropositive children. BMC Infectious Diseases, 14, 569.PubMedPubMedCentralCrossRefGoogle Scholar
  19. 19.
    Kohara, M. M., & Blum, R. N. (2006). Cytomegalovirus ileitis and hemophagocytic syndrome associated with use of anti-tumor necrosis factor-alpha antibody. Clinical Infectious Diseases, 42, 733–734.PubMedCrossRefGoogle Scholar
  20. 20.
    Bea Granell, S., Beneyto Castello, I., Ramos Escorihuela, D., Sanchez Plumed, J., Sanchez Perez, P., Hernandez-Jaras, J., et al. (2011). Cytomegalovirus-associated haemophagocytic syndrome in a kidney transplant patient. Nefrología, 31, 236–238.PubMedGoogle Scholar
  21. 21.
    Hardikar, W., Pang, K., Al-Hebbi, H., Curtis, N., & Couper, R. (2006). Successful treatment of cytomegalovirus-associated haemophagocytic syndrome following paediatric orthotopic liver transplantation. Journal of Paediatrics and Child Health, 42, 389–391.PubMedCrossRefGoogle Scholar
  22. 22.
    Amenomori, M., Migita, K., Miyashita, T., Yoshida, S., Ito, M., Eguchi, K., et al. (2005). Cytomegalovirus-associated hemophagocytic syndrome in a patient with adult onset Still's disease. Clinical and Experimental Rheumatology, 23, 100–102.PubMedGoogle Scholar
  23. 23.
    Imashuku, S., Ueda, I., Teramura, T., Mori, K., Morimoto, A., Sako, M., et al. (2005). Occurrence of haemophagocytic lymphohistiocytosis at less than 1 year of age: Analysis of 96 patients. European Journal of Pediatrics, 164, 315–319.PubMedCrossRefGoogle Scholar
  24. 24.
    Oloomi, Z., & Moayeri, H. (2006). Cytomegalovirus infection-associated hemophagocytic syndrome. Archives of Iranian Medicine, 9, 284–287.PubMedGoogle Scholar
  25. 25.
    Hot, A., Madoux, M. H., Viard, J. P., Coppere, B., & Ninet, J. (2008). Successful treatment of cytomegalovirus-associated hemophagocytic syndrome by intravenous immunoglobulins. American Journal of Hematology, 83, 159–162.PubMedCrossRefGoogle Scholar
  26. 26.
    Knorr, B., Kessler, U., Poschl, J., Fickenscher, H., & Linderkamp, O. (2007). A haemophagocytic lymphohistiocytosis (HLH)-like picture following breastmilk transmitted cytomegalovirus infection in a preterm infant. Scandinavian Journal of Infectious Diseases, 39, 173–176.PubMedCrossRefGoogle Scholar
  27. 27.
    van Langenberg, D. R., Morrison, G., Foley, A., Buttigieg, R. J., & Gibson, P. R. (2011). Cytomegalovirus disease, haemophagocytic syndrome, immunosuppression in patients with IBD: ‘A cocktail best avoided, not stirred’. Journal of Crohn's & Colitis, 5, 469–472.CrossRefGoogle Scholar
  28. 28.
    Grossman, W. J., Radhi, M., Schauer, D., Gerday, E., Grose, C., & Goldman, F. D. (2005). Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8. Blood, 106, 1203–1206.PubMedPubMedCentralCrossRefGoogle Scholar
  29. 29.
    Li, C. F., Ye, H., Liu, H., Du, M. Q., & Chuang, S. S. (2006). Fatal HHV-8-associated hemophagocytic syndrome in an HIV-negative immunocompetent patient with plasmablastic variant of multicentric Castleman disease (plasmablastic microlymphoma). The American Journal of Surgical Pathology, 30, 123–127.PubMedCrossRefGoogle Scholar
  30. 30.
    Bossini, N., Sandrini, S., Setti, G., Luppi, M., Maiorca, P., Maffei, C., et al. (2005). Successful treatment with liposomal doxorubicin and foscarnet in a patient with widespread Kaposi's sarcoma and human herpes virus 8-related, serious hemophagocytic syndrome, after renal transplantation. Giornale Italiano di Nefrologia, 22, 281–286.PubMedGoogle Scholar
  31. 31.
    Henter, J. I., Samuelsson-Horne, A., Arico, M., Egeler, R. M., Elinder, G., Filipovich, A. H., et al. (2002). Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood, 100, 2367–2373.PubMedCrossRefGoogle Scholar
  32. 32.
    Cusini, A., Gunthard, H. F., Stussi, G., Schwarz, U., Fehr, T., Grueter, E., et al. (2010). Hemophagocytic syndrome caused by primary herpes simplex virus 1 infection: Report of a first case. Infection, 38, 423–426.PubMedCrossRefGoogle Scholar
  33. 33.
    Ikumi, K., Ando, T., Katano, H., Katsuno, M., Sakai, Y., Yoshida, M., et al. (2016). HSV-2-related hemophagocytic lymphohistiocytosis in a fingolimod-treated patient with MS. Neurol Neuroimmunol Neuroinflamm, 3, e247.PubMedPubMedCentralCrossRefGoogle Scholar
  34. 34.
    Ramasamy, K., Lim, Z. Y., Savvas, M., Salisbury, J. R., Dokal, I., Mufti, G. J., et al. (2006). Disseminated herpes virus (HSV-2) infection with rhabdomyolysis and hemophagocytic lymphohistiocytosis in a patient with bone marrow failure syndrome. Annals of Hematology, 85, 629–630.PubMedCrossRefGoogle Scholar
  35. 35.
    Spinner, M. A., Ker, J. P., Stoudenmire, C. J., Fadare, O., Mace, E. M., Orange, J. S., et al. (2016). GATA2 deficiency underlying severe blastomycosis and fatal herpes simplex virus-associated hemophagocytic lymphohistiocytosis. The Journal of Allergy and Clinical Immunology, 137, 638–640.PubMedCrossRefGoogle Scholar
  36. 36.
    Mihalcea-Danciu, M., Ellero, B., Gandoin, M., Harlay, M. L., Schneider, F., & Bilbault, P. (2014). Herpes simplex hepatitis with macrophage activation syndrome in an immunocompetent patient. La Revue de Médecine Interne, 35, 823–826.PubMedCrossRefGoogle Scholar
  37. 37.
    Desideri-Vaillant, C., Exbrayat, S., Sapin-Lory, J., Lambrechts, D., Rouxel, M., & Nicolas, X. (2012). Hemophagocytic syndrome due to Herpes simplex virus after hysteroscopy. Journal de Gynécologie, Obstétrique et Biologie de la Reproduction, 41, 672–675.PubMedCrossRefGoogle Scholar
  38. 38.
    Goulding, E. A., & Barnden, K. R. (2014). Disseminated herpes simplex virus manifesting as pyrexia and cervicitis and leading to reactive hemophagocytic syndrome in pregnancy. European Journal of Obstetrics, Gynecology, and Reproductive Biology, 180, 198–199.PubMedCrossRefGoogle Scholar
  39. 39.
    Yamaguchi, K., Yamamoto, A., Hisano, M., Natori, M., & Murashima, A. (2005). Herpes simplex virus 2-associated hemophagocytic lymphohistiocytosis in a pregnant patient. Obstetrics and Gynecology, 105, 1241–1244.PubMedCrossRefGoogle Scholar
  40. 40.
    Allen, C. E., Yu, X., Kozinetz, C. A., & McClain, K. L. (2008). Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer, 50, 1227–1235.CrossRefGoogle Scholar
  41. 41.
    Gupta, A., Weitzman, S., & Abdelhaleem, M. (2008). The role of hemophagocytosis in bone marrow aspirates in the diagnosis of hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer, 50, 192–194.CrossRefGoogle Scholar
  42. 42.
    Holter, W., Goldman, C. K., Casabo, L., Nelson, D. L., Greene, W. C., & Waldmann, T. A. (1987). Expression of functional IL 2 receptors by lipopolysaccharide and interferon-gamma stimulated human monocytes. Journal of Immunology, 138, 2917–2922.Google Scholar
  43. 43.
    Seidler, S., Zimmermann, H. W., Weiskirchen, R., Trautwein, C., & Tacke, F. (2012). Elevated circulating soluble interleukin-2 receptor in patients with chronic liver diseases is associated with non-classical monocytes. BMC Gastroenterology, 12, 38.PubMedPubMedCentralCrossRefGoogle Scholar
  44. 44.
    Suzuki, N., Morimoto, A., Ohga, S., Kudo, K., Ishida, Y., Ishii, E., et al. (2009). Characteristics of hemophagocytic lymphohistiocytosis in neonates: A nationwide survey in Japan. The Journal of Pediatrics, 155, 235–238 e231.PubMedPubMedCentralCrossRefGoogle Scholar
  45. 45.
    Vladescu, I. A., Browning, W. L., & Thomsen, I. P. (2015). Massive ferritin elevation in neonatal herpes simplex virus infection: hemophagocytic lymphohistiocytosis or herpes simplex virus alone? Journal of the Pediatric Infectious Diseases Society, 4, e48–e52.PubMedCrossRefGoogle Scholar
  46. 46.
    Purtilo, D. T., Cassel, C. K., Yang, J. P., & Harper, R. (1975). X-linked recessive progressive combined variable immunodeficiency (Duncan’s disease). Lancet, 1, 935–940.PubMedPubMedCentralCrossRefGoogle Scholar
  47. 47.
    Sayos, J., Wu, C., Morra, M., Wang, N., Zhang, X., Allen, D., et al. (1998). The X-linked lymphoproliferative-disease gene product SAP regulates signals induced through the co-receptor SLAM. Nature, 395, 462–469.PubMedCrossRefGoogle Scholar
  48. 48.
    Pasic, S., Cupic, M., & Lazarevic, I. (2012). HHV-8-related hemophagocytic lymphohistiocytosis in a boy with XLP phenotype. Journal of Pediatric Hematology/Oncology, 34, 467–471.PubMedCrossRefGoogle Scholar
  49. 49.
    Snow, A. L., Marsh, R. A., Krummey, S. M., Roehrs, P., Young, L. R., Zhang, K., et al. (2009). Restimulation-induced apoptosis of T cells is impaired in patients with X-linked lymphoproliferative disease caused by SAP deficiency. The Journal of Clinical Investigation, 119, 2976–2989.PubMedPubMedCentralGoogle Scholar
  50. 50.
    Marsh, R. A., Bleesing, J. J., Chandrakasan, S., Jordan, M. B., Davies, S. M., & Filipovich, A. H. (2014). Reduced-intensity conditioning hematopoietic cell transplantation is an effective treatment for patients with SLAM-associated protein deficiency/X-linked lymphoproliferative disease type 1. Biology of Blood and Marrow Transplantation, 20, 1641–1645.PubMedCrossRefGoogle Scholar
  51. 51.
    Re, A., Facchetti, F., Borlenghi, E., Cattaneo, C., Capucci, M. A., Ungari, M., et al. (2007). Fatal hemophagocytic syndrome related to active human herpesvirus-8/Kaposi sarcoma-associated herpesvirus infection in human immunodeficiency virus-negative, non-transplant patients without related malignancies. European Journal of Haematology, 78, 361–364.PubMedCrossRefGoogle Scholar
  52. 52.
    Lerolle, N., Laanani, M., Riviere, S., Galicier, L., Coppo, P., Meynard, J. L., et al. (2016). Diversity and combinations of infectious agents in 38 adults with an infection-triggered reactive haemophagocytic syndrome: A multicenter study. Clinical Microbiology and Infection, 22, 268.e261–268.e268.CrossRefGoogle Scholar
  53. 53.
    Cattaneo, C., Oberti, M., Skert, C., Passi, A., Farina, M., Re, A., et al. (2016). Adult onset hemophagocytic lymphohistiocytosis prognosis is affected by underlying disease and coexisting viral infection: Analysis of a single institution series of 35 patients. Hematological Oncology.Google Scholar
  54. 54.
    Devecioglu, O., Anak, S., Atay, D., Aktan, P., Devecioglu, E., Ozalp, B., et al. (2009). Pediatric acute lymphoblastic leukemia complicated by secondary hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer, 53, 491–492.CrossRefGoogle Scholar
  55. 55.
    Marabelle, A., Bergeron, C., Billaud, G., Mekki, Y., & Girard, S. (2010). Hemophagocytic syndrome revealing primary HHV-6 infection. The Journal of Pediatrics, 157, 511.PubMedCrossRefGoogle Scholar
  56. 56.
    Schaballie, H., Renard, M., Vermylen, C., Scheers, I., Revencu, N., Regal, L., et al. (2013). Misdiagnosis as asphyxiating thoracic dystrophy and CMV-associated haemophagocytic lymphohistiocytosis in Shwachman-Diamond syndrome. European Journal of Pediatrics, 172, 613–622.PubMedCrossRefGoogle Scholar
  57. 57.
    Zondag, T. C., Rokx, C., van Lom, K., van den Berg, A. R., Sonneveld, P., Dik, W. A., et al. (2016). Cytokine and viral load kinetics in human herpesvirus 8-associated multicentric Castleman’s disease complicated by hemophagocytic lymphohistiocytosis. International Journal of Hematology, 103, 469–472.PubMedCrossRefGoogle Scholar
  58. 58.
    van der Werff ten Bosch, J. E., Kollen, W. J., Ball, L. M., Brinkman, D. M., Vossen, A. C., Lankester, A. C., et al. (2009). Atypical varicella zoster infection associated with hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer, 53, 226–228.CrossRefGoogle Scholar
  59. 59.
    Waddell, B., Belcher, C., & Willey, E. (2017). Cytomegalovirus induced hemophagocytic lymphocytic histiocytosis in two pediatric patients with acute lymphoblastic leukemia. IDCases, 9, 116–118.PubMedPubMedCentralCrossRefGoogle Scholar
  60. 60.
    Abdelkefi, A., Ben Jamil, W., Torjman, L., Ladeb, S., Ksouri, H., Lakhal, A., et al. (2009). Hemophagocytic syndrome after hematopoietic stem cell transplantation: a prospective observational study. International Journal of Hematology, 89, 368–373.PubMedCrossRefGoogle Scholar
  61. 61.
    Frederiksen, J. K., & Ross, C. W. (2014). Cytomegalovirus-associated hemophagocytic lymphohistiocytosis in a patient with myasthenia gravis treated with azathioprine. Blood, 123, 2290.PubMedCrossRefGoogle Scholar
  62. 62.
    Mustafa Ali, M., Ruano Mendez, A. L., & Carraway, H. E. (2017). Hemophagocytic lymphohistiocytosis in a patient with Hodgkin lymphoma and concurrent EBV, CMV, and Candida infections. Journal of Investigative Medicine High Impact Case Reports, 5, 2324709616684514.PubMedPubMedCentralCrossRefGoogle Scholar
  63. 63.
    Garcia-Montoya, L., Saenz-Tenorio, C. N., Janta, I., Menarguez, J., Lopez-Longo, F. J., Monteagudo, I., et al. (2017). Hemophagocytic lymphohistiocytosis in a patient with Sjogren’s syndrome: Case report and review. Rheumatology International, 37, 663–669.Google Scholar
  64. 64.
    Mun, J. I., Shin, S. J., Yu, B. H., Koo, J. H., Kim, D. H., Lee, K. M., et al. (2013). A case of hemophagocytic syndrome in a patient with fulminant ulcerative colitis superinfected by cytomegalovirus. The Korean Journal of Internal Medicine, 28, 352–355.PubMedPubMedCentralCrossRefGoogle Scholar
  65. 65.
    Gur, G., Cakar, N., Uncu, N., Ayar, G., Basaran, O., Taktak, A., et al. (2015). Hemophagocytic lymphohistiocytosis secondary to Varicella zoster infection in a child with Henoch-Schonlein purpura. Pediatrics International, 57, e37–e38.PubMedCrossRefGoogle Scholar
  66. 66.
    Presti, M. A., Costantino, G., Della Torre, A., Belvedere, A., Cascio, A., & Fries, W. (2011). Severe CMV-related pneumonia complicated by the hemophagocytic lymphohistiocytic (HLH) syndrome in quiescent Crohn’s colitis: Harmful cure? Inflammatory Bowel Diseases, 17, E145–E146.PubMedCrossRefGoogle Scholar
  67. 67.
    Sakamoto, O., Ando, M., Yoshimatsu, S., Kohrogi, H., Suga, M., & Ando, M. (2002). Systemic lupus erythematosus complicated by cytomegalovirus-induced hemophagocytic syndrome and colitis. Internal Medicine, 41, 151–155.PubMedCrossRefGoogle Scholar
  68. 68.
    Tanaka, Y., Seo, R., Nagai, Y., Mori, M., Togami, K., Fujita, H., et al. (2008). Systemic lupus erythematosus complicated by cytomegalovirus-induced hemophagocytic syndrome and pneumonia. Nihon Rinshō Men'eki Gakkai Kaishi, 31, 71–75.PubMedCrossRefGoogle Scholar
  69. 69.
    Luppi, M., Barozzi, P., Rasini, V., Riva, G., Re, A., Rossi, G., et al. (2002). Severe pancytopenia and hemophagocytosis after HHV-8 primary infection in a renal transplant patient successfully treated with foscarnet. Transplantation, 74, 131–132.PubMedCrossRefGoogle Scholar
  70. 70.
    Asci, G., Toz, H., Ozkahya, M., Cagirgan, S., Duman, S., Sezis, M., et al. (2006). High-dose immunoglobulin therapy in renal transplant recipients with hemophagocytic histiocytic syndrome. Journal of Nephrology, 19, 322–326.PubMedGoogle Scholar
  71. 71.
    Dharancy, S., Crombe, V., Copin, M. C., Boleslawski, E., Bocket, L., Declerck, N., et al. (2008). Fatal hemophagocytic syndrome related to human herpesvirus-6 reinfection following liver transplantation: A case report. Transplantation Proceedings, 40, 3791–3793.PubMedCrossRefGoogle Scholar
  72. 72.
    Vijgen, S., Wyss, C., Meylan, P., Bisig, B., Letovanec, I., Manuel, O., et al. (2016). Fatal outcome of multiple clinical presentations of human herpesvirus 8-related disease after solid organ transplantation. Transplantation, 100, 134–140.PubMedCrossRefGoogle Scholar
  73. 73.
    Mularoni, A., Gallo, A., Riva, G., Barozzi, P., Miele, M., Cardinale, G., et al. (2017). Successful treatment of kaposi sarcoma-associated herpesvirus inflammatory cytokine syndrome after kidney-liver transplant: Correlations with the human herpesvirus 8 miRNome and specific T cell response. American Journal of Transplantation.Google Scholar
  74. 74.
    Low, P., Neipel, F., Rascu, A., Steininger, H., Manger, B., Fleckenstein, B., et al. (1998). Suppression of HHV-8 viremia by foscarnet in an HIV-infected patient with Kaposi's sarcoma and HHV-8 associated hemophagocytic syndrome. European Journal of Medical Research, 3, 461–464.PubMedGoogle Scholar
  75. 75.
    Pastore, R. D., Chadburn, A., Kripas, C., & Schattner, E. J. (2000). Novel association of haemophagocytic syndrome with Kaposi’s sarcoma-associated herpesvirus-related primary effusion lymphoma. British Journal of Haematology, 111, 1112–1115.Google Scholar
  76. 76.
    Fardet, L., Blum, L., Kerob, D., Agbalika, F., Galicier, L., Dupuy, A., et al. (2003). Human herpesvirus 8-associated hemophagocytic lymphohistiocytosis in human immunodeficiency virus-infected patients. Clinical Infectious Diseases, 37, 285–291.PubMedCrossRefGoogle Scholar
  77. 77.
    Yates, J. A., Zakai, N. A., Griffith, R. C., Wing, E. J., & Schiffman, F. J. (2007). Multicentric Castleman disease, Kaposi sarcoma, hemophagocytic syndrome, and a novel HHV8-lymphoproliferative disorder. The AIDS Reader, 17, 596–598, 601.PubMedGoogle Scholar
  78. 78.
    Uneda, S., Murata, S., Sonoki, T., Matsuoka, H., & Nakakuma, H. (2009). Successful treatment with liposomal doxorubicin for widespread Kaposi’s sarcoma and human herpesvirus-8 related severe hemophagocytic syndrome in a patient with acquired immunodeficiency syndrome. International Journal of Hematology, 89, 195–200.Google Scholar
  79. 79.
    Uldrick, T. S., Wang, V., O’Mahony, D., Aleman, K., Wyvill, K. M., Marshall, V., et al. (2010). An interleukin-6-related systemic inflammatory syndrome in patients co-infected with Kaposi sarcoma-associated herpesvirus and HIV but without Multicentric Castleman disease. Clinical Infectious Diseases, 51, 350–358.Google Scholar
  80. 80.
    Ohkuma, K., Saraya, T., Sada, M., & Kawai, S. (2013). Evidence for cytomegalovirus-induced haemophagocytic syndrome in a young patient with AIDS. BML Case Reports, 2013.Google Scholar
  81. 81.
    Polizzotto, M. N., Uldrick, T. S., Wyvill, K. M., Aleman, K., Marshall, V., Wang, V., et al. (2016). Clinical features and outcomes of patients with symptomatic Kaposi Sarcoma Herpesvirus (KSHV)-associated inflammation: Prospective characterization of KSHV Inflammatory Cytokine Syndrome (KICS). Clinical Infectious Diseases, 62, 730–738.PubMedCrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Division of Infectious Diseases, Department of PediatricsVanderbilt University School of MedicineNashvilleUSA

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