title: Severe hemophagocytic lymphohistiocytosis in adults-experience from an intensive care unit from North India.
abstract: BACKGROUND
  Hemophagocytic lymphohistiocytosis (HLH) has been reported to complicate fulminant tropical infections but data on severe HLH with multi-organ dysfunction (MODS) are scant.
  
  
  METHODS
  Retrospective review of medical electronic records of our intensive care unit (ICU) over a 2-year period.
  
  
  RESULTS
  We describe 10 adult patients with HLH and MODS. Patients had short symptom duration prior to presentation and had rapid deterioration during their hospitalization course. Fever, organomegaly, neurologic abnormalities, hepatic abnormalities, and cutaneous signs were very common. No patient had diagnosed HLH at ICU admission (median 4 criteria [Inter Quartile Range 2-4.25]). All patients required mechanical ventilation and 80% required vasopressors. Infection-associated HLH (IAHS) was the most common etiology (80%). Seventy percent (7/10) of patients were treated with steroids and 20% received intravenous immunoglobulin. Etoposide and/or cyclosporine were administered in 20% (2/10). Nosocomial infections occurred in 40% and the ICU mortality was 70%.
  
  
  CONCLUSIONS
  Severe HLH with MODS has a very high mortality. Data on adult cohorts with IAHS in the tropics with defined treatment protocols are urgently needed.
authors.affiliation: Department of Pulmonology and Critical Care, Global Hospitals and Health City, Chennai, India.
authors.forename: Srinivas
authors.initials: S
authors.lastname: Rajagopala
authors.forename: Navneet
authors.initials: N
authors.lastname: Singh
authors.forename: Ritesh
authors.initials: R
authors.lastname: Agarwal
authors.forename: Dheeraj
authors.initials: D
authors.lastname: Gupta
authors.forename: Reena
authors.initials: R
authors.lastname: Das
keywords: Hemophagocytic lymphohistiocytosis; hemophagocytic syndrome; multi-organ dysfunction; tropics
references.citation: Hematology. 2008 Oct;13(5):286-8
references.pmid: 18854091
references.citation: Int J Hematol. 2000 Jul;72(1):1-11
references.pmid: 10979202
references.citation: Indian Pediatr. 2011 Jan;48(1):31-5
references.pmid: 20972297
references.citation: Immunol Allergy Clin North Am. 2008 May;28(2):293-313, viii
references.pmid: 18424334
references.citation: Lancet Infect Dis. 2007 Dec;7(12):814-22
references.pmid: 18045564
references.citation: APMIS. 2009 Oct;117(10):773-7
references.pmid: 19775347
references.citation: Indian Pediatr. 2000 May;37(5):526-31
references.pmid: 10820546
references.citation: Crit Rev Oncol Hematol. 2002 Dec;44(3):259-72
references.pmid: 12467966
references.citation: J Infect. 2008 May;56(5):381-8
references.pmid: 18405976
references.citation: Trop Doct. 2010 Oct;40(4):249-50
references.pmid: 20667920
references.citation: Am J Hematol. 2001 Sep;68(1):4-10
references.pmid: 11559930
references.citation: Med Pediatr Oncol. 2002 May;38(5):305-9
references.pmid: 11979453
references.citation: Blood. 1997 Jun 1;89(11):4100-3
references.pmid: 9166851
references.citation: Pediatr Blood Cancer. 2007 Feb;48(2):124-31
references.pmid: 16937360
references.citation: Hematology. 2005;10 Suppl 1:104-7
references.pmid: 16188649
references.citation: Intensive Care Med. 2010 Oct;36(10):1695-702
references.pmid: 20532477
references.citation: Lancet Infect Dis. 2006 Jul;6(7):447-54
references.pmid: 16790385
references.citation: Crit Care Med. 2004 Jun;32(6):1316-21
references.pmid: 15187513
references.citation: Intensive Care Med. 2008 Jul;34(7):1177-87
references.pmid: 18427781