Risk factors for early death in children with haemophagocytic lymphohistiocytosis

Acta Paediatrica, International Journal of Paediatrics

Volumn 101, Issue 3, 2012, Pages 313-318

  Trottestam, Helena ^a   Berglöf, Elisabet ^a   Horne, Annacarin ^a   Onelöv, Erik ^a   Beutel, Karin ^b   Lehmberg, Kai ^c   Sieni, Elena ^d   Silfverberg, Thomas ^a   Aricò, Maurizio ^d   Janka, Gritta ^c   Henter, Jan Inge ^a  

^a KAROLINSKA UNIVERSITY HOSPITAL   (Sweden)

^b UNIVERSITY HOSPITAL MÜNSTER   (Germany)

^c UNIVERSITY MEDICAL CENTER HAMBURG EPPENDORF   (Germany)

^d UNIVERSITY OF FLORENCE   (Italy)

Author keywords

FHL; Haemophagocytic lymphohistiocytosis; HLH; Risk factors

Indexed keywords

ALBUMIN; ASPARTATE AMINOTRANSFERASE; BILIRUBIN; CREATININE; CYCLOSPORIN A; DEXAMETHASONE; ETOPOSIDE; FERRITIN; FIBRINOGEN; HEMOGLOBIN; LACTATE DEHYDROGENASE; PRF1 PROTEIN; PROTEIN; STX11 PROTEIN; STXBP2 PROTEIN; UNC13D PROTEIN; UNCLASSIFIED DRUG;

ADOLESCENT; ANEMIA; ARTICLE; CHILD; CHILDHOOD DISEASE; DISEASE ASSOCIATION; FEMALE; FEVER; FOLLOW UP; GENE MUTATION; HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; HUMAN; HYPERBILIRUBINEMIA; HYPERFERRITINEMIA; INFANT; MAJOR CLINICAL STUDY; MALE; MEDICAL DECISION MAKING; PARTIAL THROMBOPLASTIN TIME; PLEOCYTOSIS; PRESCHOOL CHILD; PRIORITY JOURNAL; RISK FACTOR; SCHOOL CHILD; THROMBOCYTOPENIA;

ADOLESCENT; ANTI-INFLAMMATORY AGENTS; CHILD; CYCLOSPORINE; DEXAMETHASONE; DRUG THERAPY, COMBINATION; ETOPOSIDE; FEMALE; FERRITINS; FEVER; HUMANS; HYPERBILIRUBINEMIA; IMMUNOSUPPRESSIVE AGENTS; LEUKOCYTOSIS; LYMPHOHISTIOCYTOSIS, HEMOPHAGOCYTIC; MALE; PROGNOSIS; PROPORTIONAL HAZARDS MODELS; RISK FACTORS; THROMBOCYTOPENIA;

EID: 84856333405     PISSN: 08035253     EISSN: 16512227     Source Type: Journal    
DOI: 10.1111/j.1651-2227.2011.02501.x     Document Type: Article

References (30)

1. Janka G,. Hemophagocytic lymphohistiocytosis: when the immune system runs amok. Klin Pädiatr 2009; 221: 278-85.
2. Henter JI, Horne A, Aricò M, Egeler RM, Filipovich AH, Imashuku S, et al., HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48: 124-31. (Pubitemid 44958267)
3. Imashuku S,. Differential diagnosis of hemophagocytic syndrome: underlying disorders and selection of the most effective treatment. Int J Hematol 1997; 66: 135-51. (Pubitemid 27360177)
4. Imashuku S, Hibi S, Ohara T, Iwai A, Sako M, Kato M, et al., Effective control of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis with immunochemotherapy. Blood 1999; 93: 1869-74. (Pubitemid 29128488)
5. Stepp SE, Dufourcq-Lagelouse R, Le Deist F, Bhawan S, Certain S, Mathew PA, et al., Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science 1999; 286: 1957-9. (Pubitemid 129515904)
6. Feldmann J, Callebaut I, Raposo G, Certain S, Bacq D, Dumont C, et al., Munc 13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3). Cell 2003; 115: 461-73. (Pubitemid 37456808)
7. zur Stadt U, Schmidt S, Kasper B, Beutel K, Diler AS, Henter JI, et al., Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type-4 to chromosome 6q24 and identification of mutations in syntaxin 11. Hum Mol Genet 2005; 14: 827-34. (Pubitemid 40403278)
8. Côte M, Ménager M, Burgess A, Mahlaoui N, Picard C, Schaffner C, et al., Munc18-2 deficiency causes hemophagocytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cells. J Clin Invest 2009; 119: 3765-73.
9. zur Stadt U, Rohr J, Seifert W, Koch F, Grieve S, Pagel J, et al., Familial hemophagocytic lymphohistiocytosis type 5 (FHL5) is caused by mutations in Munc18-2 and impaired binding to syntaxin 11. Am J Hum Genet 2009; 85: 482-92.
10. Henter JI, Samuelsson-Horne A, Aricò M, Egeler RM, Filipovich AH, Imashuku S, et al., Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002; 100: 2367-73.
11. Janka GE,. Familial hemophagocytic lymphohistiocytosis. Eur J Pediatr 1983; 40: 221-30. (Pubitemid 13026976)
12. Pachlopnik Schmid J, Moshous D, Boddaert N, Neven B, Dal Cortivo L, Tardieu M, et al., Hematopoietic stem cell transplantation in Griscelli syndrome type 2: a single-center report on 10 patients. Blood 2009; 11: 211-8.
13. Pachlopnik Schmid J, Canioni D, Moshous D, Touzot F, Mahlaoui N, Hauck F, et al., Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency). Blood 2001; 2: 1522-9.
14. Rihani R, Barbar M, Faqih N, Halalsheh H, Hussein AA, Al-Zaben AH, et al., Unrelated cord blood transplantation can restore hematologic and immunologic functions in patients with Chediak-Higashi Syndrome. Pediatr Transplant 2011; (Epub) DOI:.
15. Enders A, Zieger B, Schwarz K, Yoshimi A, Speckmann C, Knoepfle EM, et al., Lethal hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type II. Blood 2006; 108: 81-7. (Pubitemid 43990615)
16. Shabbir M, Lucas J, Lazarchick J, Shirai K,. Secondary hemophagocytic syndrome in adults: a case series of 18 patients in a single institution and a review of the literature. Hematol Oncol 2011; 29: 100-6.
17. Imashuku S,. Treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH); update 2010. J Pediatr Hematol Oncol 2011; 33: 35-9.
18. Machaczka M, Vaktnäs J, Klimkowska M, Hägglund H,. Malignancy-associated hemophagocytic lymphohistiocytosis in adults: a retrospective population-based analysis from a single center. Leuk Lymphoma 2011; 2: 613-9.
19. Gupta AA, Tyrrel P, Valani R, Benseler S, Abdelhaleem M, Weitzman S,. Experience with hemophagocytic lymphohistiocytosis/macrophage activating syndrome at a single institution. J Pediatr Hematol Oncol 2009; 31: 81-4.
20. Horne A, Trottestam H, Aricò M, Egeler M, Filipovich AH, Gadner H, et al., Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis. Br J Haematol 2008; 140: 327-35.
21. Janka GE,. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr 2007; 166: 95-109. (Pubitemid 46025500)
22. Mercenaro S, Gallo F, Martini S, Santoro A, Griffiths GM, Aricò M, et al., Analysis of natural killer-cell function in familial hemophagocytic lymphohistiocytosis (FHL): defective CD107a surface expression heralds Munc13-4 defect and discriminates between genetic subtypes of the disease. Blood 2006; 108: 2316-23. (Pubitemid 44497515)
23. Kogawa K, Lee SM, Villanueva J, Marmer D, Sumegi J, Filipovich AH,. Perforin expression in cytotoxic lymphocytes from patients with hemophagocytic lymphohistiocytosis and their family members. Blood 2002; 9: 61-6.
24. Bryceson YT, Rudd E, Zheng C, Edner J, Ma D, Wood SM, et al., Defective cytotoxic lymphocyte degranulation in syntaxin 11-deficient familial hemophagocytic lymphohistiocytosis 4 (FHL4) patients. Blood 2007; 110: 1906-15. (Pubitemid 47443904)
25. Meeths M, Entesarian M, Al-Herz W, Chiang SC, Wood SM, Al-Ateegi W, et al., Spectrum of clinical presentations in familial hemophagocytic lymphohistiocytosis type 5 patients with mutations in STXBP2. Blood 2010; 116: 2635-43.
26. Kliegman RM, Behrman RE, Jenson HB, Stanton BF, editors. Nelson textbook of pediatrics, 18th ed. London: WB Saunders & Co, 2007.
27. Aricò M, Janka G, Fischer A, Henter JI, Blanche S, Elinder G, et al., Hemophagocytic lymphohistiocytosis. Report of 122 children from the international registry. Leukemia 1996; 10: 197-203. (Pubitemid 26102881)
28. Imashuku S, Hibi S, Todo S,. Hemophagocytic lymphohistiocytosis in infancy and childhood. J Pediatr 1997; 130: 352-7. (Pubitemid 27492677)
29. Lin TF, Ferlic-Stark LL, Allen CE, Kozinetz CA, McClain KL,. Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality. Pediatr Blood Cancer 2011; 56: 154-5.
30. Trottestam H, Horne AC, Aricò M, Egeler RM, Filipovich AH, Gadner H, et al., Chemo-immunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood 2011; 118: 4577-84.