What intensivists need to know about hemophagocytic syndrome: An underrecognized cause of death in adult intensive care units

Journal of Intensive Care Medicine

Volumn 27, Issue 1, 2012, Pages 58-64

  Okabe, Toshimasa ^a   Shah, Gunjan ^a   Mendoza, Vinia ^a   Hirani, Amyn ^a   Baram, Michael ^a   Marik, Paul ^b  

^a THOMAS JEFFERSON UNIVERSITY   (United States)

^b EASTERN VIRGINIA MEDICAL SCHOOL   (United States)

Author keywords

ductopenia; hemophagocytic lymphohistiocytosis; hemophagocytic syndrome; hyperferritinemia; systemic inflammatory response syndrome

Indexed keywords

ALPRAZOLAM; COLESTYRAMINE; CYCLOSPORIN; CYCLOSPORIN A; DACLIZUMAB; DEXAMETHASONE; DIGOXIN; DIPHENHYDRAMINE; ESOMEPRAZOLE; ETOPOSIDE; GUAIFENESIN; HYDROXYZINE; IMMUNOGLOBULIN; INFLIXIMAB; METHOTREXATE; METHYLPREDNISOLONE; METOPROLOL; OMEPRAZOLE; PREDNISONE; WARFARIN;

ADULT; AGED; ARTICLE; BIOPSY; CASE REPORT; CAUSE OF DEATH; CLINICAL FEATURE; DISEASE COURSE; DRUG MEGADOSE; FEMALE; FERRITIN BLOOD LEVEL; HEMOPHAGOCYTIC SYNDROME; HUMAN; IMMUNE RESPONSE; INTENSIVE CARE UNIT; MALE; PRIORITY JOURNAL;

ADULT; AGED; ATRIAL FIBRILLATION; CLINICAL COMPETENCE; COMBINED MODALITY THERAPY; DIABETES MELLITUS; DIAGNOSIS, DIFFERENTIAL; FATIGUE; FEMALE; FEVER; HUMANS; HYPERLIPIDEMIAS; INTENSIVE CARE; INTENSIVE CARE UNITS; INTERNAL MEDICINE; LIVER DISEASES; LYMPHOHISTIOCYTOSIS, HEMOPHAGOCYTIC; MALE; MIDDLE AGED; MULTIPLE ORGAN FAILURE; NEOPLASMS; PATIENT ADMISSION; SARCOIDOSIS; SERUM;

EID: 84856159580     PISSN: 08850666     EISSN: 15251489     Source Type: Journal    
DOI: 10.1177/0885066610393462     Document Type: Article

References (35)

1. Farquhar JW, Claireaux AE.. Familial haemophagocytic reticulosis. Arch Dis Child. 1952 ; 27 (136). 519-525
2. Arico M, Janka G, Fischer A, et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia. 1996 ; 10 (2). 197-203 (Pubitemid 26102881)
3. Allen M, De Fusco C, Legrand F, et al. Familial hemophagocytic lymphohistiocytosis: how late can the onset be?. Haematologica. 2001 ; 86 (5). 499-503 (Pubitemid 33638978)
4. Grom AA.. Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities?. Curr Opin Rheumatol. 2003 ; 15 (5). 587-590 (Pubitemid 37041397)
5. Henter JI, Horne A, Arico M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007 ; 48 (2). 124-131 (Pubitemid 44958267)
6. Spivack T, Chawla R, Marik PE.. Epstein-Barr virus-associated hemophagocytic syndrome mimicking severe sepsis. J Emerg Trauma Shock. 2008 ; 1 (2). 119-122
7. Henter JI, Elinder G, Soder O, Ost A.. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand. 1991 ; 80 (4). 428-435
8. Strauss R, Neureiter D, Westenburger B, Wehler M, Kirchner T, Hahn EG.. Multifactorial risk analysis of bone marrow histiocytic hyperplasia with hemophagocytosis in critically ill medical patients-a postmortem clinicopathologic analysis. Crit Care Med. 2004 ; 32 (6). 1316-1321 (Pubitemid 38738586)
9. Filipovich AH.. Hemophagocytic lymphohistiocytosis and other hemophagocytic disorders. Immunol Allergy Clin North Am. 2008 ; 28 (2). 293-313, viii (Pubitemid 351522416)
10. Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C.. Infections associated with haemophagocytic syndrome. Lancet Infect Dis. 2007 ; 7 (12). 814-822 (Pubitemid 350152044)
11. Imashuku S.. Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Crit Rev Oncol Hematol. 2002 ; 44 (3). 259-272 (Pubitemid 35435291)
12. Tabata YS, Teramura T, Kuriyama K, et al. Molecular analysis of latent membrane protein 1 in patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in Japan. Leuk Lymphoma. 2000 ; 38 (3-4). 373-380 (Pubitemid 30303671)
13. Chuang HC, Lay JD, Hsieh WC, Su IJ.. Pathogenesis and mechanism of disease progression from hemophagocytic lymphohistiocytosis to Epstein-Barr virus-associated T-cell lymphoma: nuclear factor-kappa B pathway as a potential therapeutic target. Cancer Sci. 2007 ; 98 (9). 1281-1287
14. Chuang HC, Lay JD, Hsieh WC, et al. Epstein-Barr virus LMP1 inhibits the expression of SAP gene and upregulates Th1 cytokines in the pathogenesis of hemophagocytic syndrome. Blood. 2005 ; 106 (9). 3090-3096
15. Chuang HC, Lay JD, Chuang SE, Hsieh WC, Chang Y, Su IJ.. Epstein-Barr virus (EBV) latent membrane protein-1 down-regulates tumor necrosis factor-alpha (TNF-alpha) receptor-1 and confers resistance to TNF-alpha-induced apoptosis in T cells: implication for the progression to T-cell lymphoma in EBV-associated hemophagocytic syndrome. Am J Pathol. 2007 ; 170 (5). 1607-1617 (Pubitemid 47339305)
16. Goransdotter Ericson K, Fadeel B, Nilsson-Ardnor S, et al. Spectrum of perforin gene mutations in familial hemophagocytic lymphohistiocytosis. Am J Hum Genet. 2001 ; 68 (3). 590-597 (Pubitemid 32202754)
17. Clementi R, zur Stadt U, Savoldi G, et al. Six novel mutations in the PRF1 gene in children with haemophagocytic lymphohistiocytosis. J Med Genet. 2001 ; 38 (9). 643-646 (Pubitemid 32835632)
18. Feldmann J, Callebaut I, Raposo G, et al. Munc13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3). Cell. 2003 ; 115 (4). 461-473 (Pubitemid 37456808)
19. zur Stadt U, Schmidt S, Kasper B, 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 (6). 827-834 (Pubitemid 40403278)
20. Henter JI.. Pronounced hyperferritinemia: expanding the field of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008 ; 50 (6). 1127-1129
21. Esumi N, Ikushima S, Todo S, Imashuku S.. Hyperferritinemia in malignant histiocytosis, virus-associated hemophagocytic syndrome and familial erythrophagocytic lymphohistiocytosis. A survey of pediatric cases. Acta Paediatr Scand. 1989 ; 78 (2). 268-270 (Pubitemid 19079576)
22. Allen CE, Yu X, Kozinetz CA, McClain KL.. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008 ; 50 (6). 1227-1235 (Pubitemid 351555548)
23. Thomas M, Jankovic J.. Neurodegenerative disease and iron storage in the brain. Curr Opin Neurol. 2004 ; 17 (4). 437-442 (Pubitemid 39071420)
24. You SA, Wang Q.. Ferritin in atherosclerosis. Clin Chim Acta. 2005 ; 357 (1). 1-16 (Pubitemid 40814431)
25. Kabat GC, Rohan TE.. Does excess iron play a role in breast carcinogenesis? An unresolved hypothesis. Cancer Causes Control. 2007 ; 18 (10). 1047-1053 (Pubitemid 47498168)
26. Knovich MA, Storey JA, Coffman LG, Torti SV, Torti FM.. Ferritin for the clinician. Blood Rev. 2009 ; 23 (3). 95-104
27. Creput C, Galicier L, Buyse S, Azoulay E.. Understanding organ dysfunction in hemophagocytic lymphohistiocytosis. Intensive Care Med. 2008 ; 34 (7). 1177-1187
28. Kikuta H, Sakiyama Y, Matsumoto S, et al. Fatal Epstein-Barr virus-associated hemophagocytic syndrome. Blood. 1993 ; 82 (11). 3259-3264 (Pubitemid 23356693)
29. Balta G, Okur H, Unal S, et al. Assessment of clinical and laboratory presentations of familial hemophagocytic lymphohistiocytosis patients with homozygous W374X mutation. Leuk Res. 2010 ; 34 (8). 1012-1017
30. Okamoto M, Yamaguchi H, Isobe Y, et al. Analysis of triglyceride value in the diagnosis and treatment response of secondary hemophagocytic syndrome. Intern Med. 2009 ; 48 (10). 775-781
31. Reau NS, Jensen DM.. Vanishing bile duct syndrome. Clin Liver Dis. 2008 ; 12 (1). 203-217, x (Pubitemid 351175655)
32. Kikuchi K, Miyakawa H, Abe K, et al. Vanishing bile duct syndrome associated with chronic EBV infection. Dig Dis Sci. 2000 ; 45 (1). 160-165 (Pubitemid 30111703)
33. Imashuku S, Kuriyama K, Sakai R, et al. Treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in young adults: a report from the HLH study center. Med Pediatr Oncol. 2003 ; 41 (2). 103-109 (Pubitemid 36858327)
34. Tomaske M, Amon O, Bosk A, Handgretinger R, Schneider EM, Niethammer D.. Alpha-CD25 antibody treatment in a child with hemophagocytic lymphohistiocytosis. Med Pediatr Oncol. 2002 ; 38 (2). 141-142 (Pubitemid 34113453)
35. Henzan T, Nagafuji K, Tsukamoto H, et al. Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis. Am J Hematol. 2006 ; 81 (1). 59-61 (Pubitemid 43077214)