Therapeutic role of anakinra, an interleukin-1 receptor antagonist, in the management of secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction/macrophage activating syndrome in critically Ill children

Pediatric Critical Care Medicine

Volumn 15, Issue 5, 2014, Pages 401-408

  Rajasekaran, Surender ^a   Kruse, Katherine ^a,b   Kovey, Karen ^a   Davis, Alan T ^b   Hassan, Nabil E ^a   Ndika, Akunne N ^a   Zuiderveen, Sandra ^a   Birmingham, James ^a  

^a HELEN DEVOS CHILDREN S HOSPITAL   (United States)

^b Grand Rapids Medical Education Partners   (United States)

Author keywords

Cytokines; Hemophagocytic lymphohistiocytosis; Inflammation; Macrophage activating syndrome; Multiple organ dysfunction syndrome; Pharmacotherapy

Indexed keywords

C REACTIVE PROTEIN; CORTICOSTEROID; CREATININE; FERRITIN; FIBRINOGEN; IMMUNOGLOBULIN; LACTATE DEHYDROGENASE; RECOMBINANT INTERLEUKIN 1 RECEPTOR BLOCKING AGENT; TRIACYLGLYCEROL; ANTIINFLAMMATORY AGENT; ANTIRHEUMATIC AGENT; IMMUNOLOGIC FACTOR; INTERLEUKIN 1 RECEPTOR BLOCKING AGENT; STEROID;

ADOLESCENT; ADULT; ARTICLE; CARDIOVASCULAR DISEASE; CASE STUDY; CHILD; CHILDHOOD MORTALITY; CLINICAL ARTICLE; CORTICOSTEROID THERAPY; CRITICALLY ILL PATIENT; CYTOPENIA; DRUG MEGADOSE; DRUG WITHDRAWAL; FEMALE; FERRITIN BLOOD LEVEL; HEMATOPOIETIC STEM CELL TRANSPLANTATION; HEMOPHAGOCYTIC SYNDROME; HUMAN; HUMAN TISSUE; HYPERTRANSAMINASEMIA; INTENSIVE CARE UNIT; LYMPHOCYTE COUNT; MACROPHAGE ACTIVATION SYNDROME; MALE; MULTIPLE ORGAN FAILURE; NEUTROPHIL COUNT; PLEOCYTOSIS; PRIORITY JOURNAL; RESPIRATORY DISTRESS; RETROSPECTIVE STUDY; SCHOOL CHILD; SCORING SYSTEM; SEPSIS; THORAX PAIN; THROMBOCYTE COUNT; TREATMENT INDICATION; YOUNG ADULT; BLOOD; INTENSIVE CARE; LYMPHOCYTE; LYMPHOHISTIOCYTOSIS, HEMOPHAGOCYTIC; METABOLISM; NEUTROPHIL;

ADOLESCENT; ANTI-INFLAMMATORY AGENTS; ANTIRHEUMATIC AGENTS; C-REACTIVE PROTEIN; CHILD; FEMALE; FERRITINS; FIBRINOGEN; HUMANS; IMMUNOGLOBULINS, INTRAVENOUS; IMMUNOLOGIC FACTORS; INTENSIVE CARE; INTERLEUKIN 1 RECEPTOR ANTAGONIST PROTEIN; LYMPHOCYTE COUNT; LYMPHOCYTES; LYMPHOHISTIOCYTOSIS, HEMOPHAGOCYTIC; MACROPHAGE ACTIVATION SYNDROME; MALE; MULTIPLE ORGAN FAILURE; NEUTROPHILS; RETROSPECTIVE STUDIES; SEPSIS; STEROIDS; YOUNG ADULT;

EID: 84902271890     PISSN: 15297535     EISSN: 19473893     Source Type: Journal    
DOI: 10.1097/PCC.0000000000000078     Document Type: Article

References (32)

1. Henter JI, Aricò M, Egeler RM, et al: HLH-94: A treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society. Med Pediatr Oncol 1997; 28:342-347
2. Janka GE: Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr 2007; 166:95-109
3. Castillo L, Carcillo J: Secondary hemophagocytic lymphohistiocytosis and severe sepsis/systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation. Pediatr Crit Care Med 2009; 10:387-392
4. Raschke RA, Garcia-Orr R: Hemophagocytic lymphohistiocytosis: A potentially underrecognized association with systemic inflammatory response syndrome, severe sepsis, and septic shock in adults. Chest 2011; 140:933-938
5. Demirkol D, Yildizdas D, Bayrakci B, et al: Hyperferritinemia in the critically ill child with secondary HLH/sepsis/MODS/MAS: What is the treatment? Crit Care 2012; 16:R52
6. Risma K, Jordan MB: Hemophagocytic lymphohistiocytosis: Updates and evolving concepts. Curr Opin Pediatr 2012; 24:9-15
7. Henter JI, Horne A, Arico M, et al: HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124-131
8. Jordan MB, Allen CE, Weitzman S, et al: How I treat hemophagocytic lymphohistiocytosis. Blood 2011; 118:4041-4052
9. Ishii E, Ohga S, Aoki T, et al: Prognosis of children with virus- associated hemophagocytic syndrome and malignant histiocytosis: Correlation with levels of serum interleukin-1 and tumor necrosis factor. Acta Haematol 1991; 85:93-99
10. Bruck N, Suttorp M, Kabus M, et al: Rapid and sustained remission of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome through treatment with anakinra and corticosteroids. J Clin Rheumatol 2011; 17:23-27
11. Miettunen PM, Narendran A, Jayanthan A, et al: Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: Case series with 12 patients. Rheumatology (Oxford) 2011; 50:417-419
12. Wilkinson JD, Pollack MM, Ruttimann UE, et al: Outcome of pediatric patients with multiple organ system failure. Crit Care Med 1986; 14:271-274
13. Proulx F, Fayon M, Farrell CA, et al: Epidemiology of sepsis and multiple organ dysfunction syndrome in children. Chest 1996; 109:1033-1037
14. Nigrovic PA, Mannion M, Prince FH, et al: 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; 63:545-555
15. Opal SM, Fisher CJ Jr, Dhainaut JF, et al: Confirmatory interleukin-1 receptor antagonist trial in severe sepsis: A phase III, randomized, double-blind, placebo-controlled, multicenter trial. The Interleukin-1 Receptor Antagonist Sepsis Investigator Group. Crit Care Med 1997; 25:1115-1124
16. Henter JI, Samuelsson-Horne A, Aricò M, et al; Histocyte Society: Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002; 100:2367-2373
17. Janka GE: Familial and acquired hemophagocytic lymphohistiocytosis. Annu Rev Med 2012; 63:233-246
18. Dinarello CA: Interleukin-1 in the pathogenesis and treatment of inflammatory diseases. Blood 2011; 117:3720-3732
19. Goodman RB, Strieter RM, Martin DP, et al: Inflammatory cytokines in patients with persistence of the acute respiratory distress syndrome. Am J Respir Crit Care Med 1996; 154:602-611
20. Hybertson BM, Lee YM, Repine JE: Phagocytes and acute lung injury: Dual roles for interleukin-1. Ann N Y Acad Sci 1997; 832:266-273
21. Meduri GU, Headley S, Kohler G, et al: Persistent elevation of inflammatory cytokines predicts a poor outcome in ARDS. Plasma IL-1 beta and IL-6 levels are consistent and efficient predictors of outcome over time. Chest 1995; 107:1062-1073
22. Pino-Yanes M, Ma SF, Sun X, et al: Interleukin-1 receptor-associated kinase 3 gene associates with susceptibility to acute lung injury. Am J Respir Cell Mol Biol 2011; 45:740-745
23. Fujiwara F, Hibi S, Imashuku S: Hypercytokinemia in hemophagocytic syndrome. Am J Pediatr Hematol Oncol 1993; 15:92-98
24. Henter JI, Andersson B, Elinder G, et al: Elevated circulating levels of interleukin-1 receptor antagonist but not IL-1 agonists in hemophagocytic lymphohistiocytosis. Med Pediatr Oncol 1996; 27:21-25
25. Fall N, Barnes M, Thornton S, et al: Gene expression profiling of peripheral blood from patients with untreated new-onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome. Arthritis Rheum 2007; 56:3793-3804
26. Sung L, Weitzman SS, Petric M, et al: The role of infections in primary hemophagocytic lymphohistiocytosis: A case series and review of the literature. Clin Infect Dis 2001; 33:1644-1648
27. Créput C, Galicier L, Buyse S, et al: Understanding organ dysfunction in hemophagocytic lymphohistiocytosis. Intensive Care Med 2008; 34:1177-1187
28. Diaz-Guzman E, Dong B, Hobbs SB, et al: Hemophagocytic lymphohistiocytosis after lung transplant: Report of 2 cases and a literature review. Exp Clin Transplant 2011; 9:217-222
29. Karras A, Thervet E, Legendre C; Groupe Coopératif de transplantation d'Ile de France: Hemophagocytic syndrome in renal transplant recipients: Report of 17 cases and review of literature. Transplantation 2004; 77:238-243
30. Stéphan JL, Koné-Paut I, Galambrun C, et al: Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology (Oxford) 2001; 40:1285-1292
31. Gupta A, Tyrrell P, Valani R, et al: The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2008; 51:402-404
32. Strauss R, Neureiter D, Westenburger B, et al: Multifactorial risk analysis of bone marrow histiocytic hyperplasia with hemophagocytosis in critically ill medical patients-A postmortem clinicopathologic analysis. Crit Care Med 2004; 32:1316-1321