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Fever and Abdominal Pain

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References

  1. Grom AA, Mellins ED. Macrophage activation syndrome: advances towards understanding pathogenesis. Curr Opin Rheumatol. 2010;22(5):561–6.

    Article  Google Scholar 

  2. Atteritano M, David A, Bagnato G, Beninati C, Frisina A, Iaria C, Cascio A. Haemophagocytic syndrome in rheumatic patients. A systematic review. Eur Rev Med Pharmacol Sci. 2012;16(10):1414–24.

    CAS  PubMed  Google Scholar 

  3. Wong K-F, Hui P-K, Chan JK, Chan Y-W, Ha S-Y. The acute lupus hemophagocytic syndrome. Ann Intern Med. 1991;114(5):387–90.

    Article  CAS  Google Scholar 

  4. Voskoboinik I, Sutton VR, Ciccone A, House CM, Chia J, Darcy PK, Yagita H, Trapani JA. Perforin activity and immune homeostasis: the common A91V polymorphism in perforin results in both presynaptic and postsynaptic defects in function. Blood. 2007;110(4):1184–90.

    Article  CAS  Google Scholar 

  5. Behrens EM, Beukelman T, Paessler M, Cron RQ. Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis. J Rheumatol. 2007;34(5):1133–8.

    PubMed  Google Scholar 

  6. Sawhney S, Woo P, Murray K. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child. 2001;85(5):421–6.

    Article  CAS  Google Scholar 

  7. Stephan J, Koné-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM. Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology. 2001;40(11):1285–92.

    Article  CAS  Google Scholar 

  8. Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124–31.

    Article  Google Scholar 

  9. Zur Stadt U, Beutel K, Kolberg S, Schneppenheim R, Kabisch H, Janka G, Hennies HC. Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis: molecular and functional analyses of PRF1, UNC13D, STX11, and RAB27A. Hum Mutat. 2006;27(1):62–8.

    Article  CAS  Google Scholar 

  10. Ravelli A. Macrophage activation syndrome. Curr Opin Rheumatol. 2002;14(5):548–52.

    Article  CAS  Google Scholar 

  11. Jenkins MR, Rudd-Schmidt JA, Lopez JA, Ramsbottom KM, Mannering SI, Andrews DM, Voskoboinik I, Trapani JA. Failed CTL/NK cell killing and cytokine hypersecretion are directly linked through prolonged synapse time. J Exp Med. 2015;212(3):307–17.

    Article  CAS  Google Scholar 

  12. Janka G. Hemophagocytic lymphohistiocytosis: when the immune system runs amok. Klin Padiatr. 2009;221(05):278–85.

    Article  CAS  Google Scholar 

  13. Grom AA. Natural killer cell dysfunction: a common pathway in systemic-onset juvenile rheumatoid arthritis, macrophage activation syndrome, and hemophagocytic lymphohistiocytosis? Arthritis Rheum. 2004;50(3):689–98.

    Article  Google Scholar 

  14. Saltzman RW, Monaco-Shawver L, Zhang K, Sullivan KE, Filipovich AH, Orange JS. Novel mutation in syntaxin-binding protein 2 (STXBP2) prevents IL-2-induced natural killer cell cytotoxicity. J Allergy Clin Immunol. 2012;129(6):1666.

    Article  CAS  Google Scholar 

  15. Spessott WA, Sanmillan ML, McCormick ME, Patel N, Villanueva J, Zhang K, Nichols KE, Giraudo CG. Hemophagocytic lymphohistiocytosis caused by dominant-negative mutations in STXBP2 that inhibit SNARE-mediated membrane fusion. Blood. 2015;125(10):1566–77.

    Article  CAS  Google Scholar 

  16. Zhang M, Bracaglia C, Prencipe G, Bemrich-Stolz CJ, Beukelman T, Dimmitt RA, Chatham WW, Zhang K, Li H, Walter MR, De Benedetti F, Grom AA, Cron RQ. A Heterozygous RAB27A mutation associated with delayed cytolytic granule polarization and hemophagocytic lymphohistiocytosis. J Immunol. 2016;196:2492–503.

    Google Scholar 

  17. Zhang X-Y, Ye X-W, Feng D-X, Han J, Li D, Zhang C. Hemophagocytic lymphohistiocytosis induced by severe pandemic influenza A (H1N1) 2009 virus infection: a case report. Case Rep Med. 2011;2011:951910.

    Article  Google Scholar 

  18. Sullivan KE, Delaat CA, Douglas SD, Filipovich AH. Defective natural killer cell function in patients with hemophagocytic lymphohistiocytosis and in first degree relatives. Pediatr Res. 1998;44(4):465–8.

    Article  CAS  Google Scholar 

  19. Villanueva J, Lee S, Giannini EH, Graham TB, Passo MH, Filipovich A, Grom AA. Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome. Arthritis Res Ther. 2004;7(1):1.

    Google Scholar 

  20. Caiello I, Minnone G, Holzinger D, Vogl T, Prencipe G, Manzo A, De Benedetti F, Strippoli R. IL-6 amplifies TLR mediated cytokine and chemokine production: implications for the pathogenesis of rheumatic inflammatory diseases. PLoS One. 2014;9(10):e107886.

    Article  Google Scholar 

  21. Zhang M, Behrens EM, Atkinson TP, Shakoory B, Grom AA, Cron RQ. Genetic defects in cytolysis in macrophage activation syndrome. Curr Rheumatol Rep. 2014;16(9):439.

    Article  Google Scholar 

  22. Schulert GS, Grom AA. Macrophage activation syndrome and cytokine-directed therapies. Best Pract Res Clin Rheumatol. 2014;28(2):277–92.

    Article  Google Scholar 

  23. Weaver LK, Behrens EM. Hyperinflammation, rather than hemophagocytosis, is the common link between macrophage activation syndrome and hemophagocytic lymphohistiocytosis. Curr Opin Rheumatol. 2014;26(5):562.

    Article  CAS  Google Scholar 

  24. Chen C, Huang Y, Jaing T, Hung I, Yang C, Chang L, Lin T. Hemophagocytic syndrome: a review of 18 pediatric cases. J Microbiol Immunol Infect. 2004;37(3):157.

    PubMed  Google Scholar 

  25. Elazary AS, Wolf DG, Amir G, Avni B, Rund D, Yehuda DB, Sviri S. Severe Epstein–Barr virus-associated hemophagocytic syndrome in six adult patients. J Clin Virol. 2007;40(2):156–9.

    Article  Google Scholar 

  26. Balamuth NJ, Nichols KE, Paessler M, Teachey DT. Use of rituximab in conjunction with immunosuppressive chemotherapy as a novel therapy for Epstein Barr virus-associated hemophagocytic lymphohistiocytosis. J Pediatr Hematol Oncol. 2007;29(8):569–73.

    Article  Google Scholar 

  27. Milone MC, Tsai DE, Hodinka RL, Silverman LB, Malbran A, Wasik MA, Nichols KE. Treatment of primary Epstein-Barr virus infection in patients with X-linked lymphoproliferative disease using B-cell–directed therapy. Blood. 2005;105(3):994–6.

    Article  CAS  Google Scholar 

  28. Henter J-I, Samuelsson-Horne A, Arico M, Egeler RM, Elinder G, Filipovich AH, Gadner H, Imashuku S, Komp D, Ladisch S. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002;100(7):2367–73.

    Article  CAS  Google Scholar 

  29. Bruck N, Suttorp M, Kabus M, Heubner G, Gahr M, Pessler F. Rapid and sustained remission of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome through treatment with anakinra and corticosteroids. J Clin Rheumatol. 2011;17(1):23–7.

    Article  Google Scholar 

  30. Kelly A, Ramanan AV. A case of macrophage activation syndrome successfully treated with anakinra. Nat Clin Pract Rheumatol. 2008;4(11):615–20.

    Article  CAS  Google Scholar 

  31. Nigrovic PA, Mannion M, Prince FH, Zeft A, Rabinovich CE, Van Rossum MA, Cortis E, Pardeo M, Miettunen PM, Janow G. Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: report of forty-six patients from an international multicenter series. Arthritis Rheum. 2011;63(2):545–55.

    Article  CAS  Google Scholar 

  32. Record JL, Beukelman T, Cron RQ. Combination therapy of abatacept and anakinra in children with refractory systemic juvenile idiopathic arthritis: a retrospective case series. J Rheumatol. 2011;38(1):180–1.

    Article  Google Scholar 

  33. Cron RQ, Chatham WW. Development of spondyloarthropathy following episodes of macrophage activation syndrome in children with heterozygous mutations in haemophagocytic lymphohistiocytosis-associated genes. Clin Exp Rheumatol. 2016;34(5):953.

    PubMed  Google Scholar 

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Eloseily, E.M., Cron, R.Q. (2020). Fever and Abdominal Pain. In: Rahmani, F., Rezaei, N. (eds) Pediatric Autoimmunity and Transplantation. Springer, Cham. https://doi.org/10.1007/978-3-030-26280-8_49

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  • DOI: https://doi.org/10.1007/978-3-030-26280-8_49

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