Características clínicas y mortalidad de pacientes adultos con síndrome hemafagocítico, estudio de cohorte retrospectiva / Hemophagocytic lymphohistiocytosis: experience in 27 patients
Rev. méd. Chile
;
145(3): 344-350, Mar. 2017. ilus, graf, tab
Article
in Spanish
| LILACS
| ID: biblio-845546
ABSTRACT
Background:
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activationAim:
To describe the clinical characteristics, causes and survival associated with HLH. Material andMethods:
Review of medical records of patients with HLH attended between 2004 and 2016. They were classified according to their probable cause in associated with immunosuppression, cancer, post-infectious or idiopathic. Kaplan-Meier survival analysis was performed.Results:
Twenty seven patients with HLH aged 18 to 87 years (59% men), were detected. Fourteen (52%) were secondary to immunosuppression, six (22%) were post-infectious, five (18%) were associated with cancer and two (7%) were of unknown cause. There were no significant differences in clinical or laboratory features between these etiologies. Within the immunosuppressed group, 12 (86%) were patients with oncologic or hematologic diseases or bone marrow transplantation. Associated cancers were mostly oncohematologic diseases. Thirty-day mortality was 53.4% (95% confidence intervals (CI) 32.7-70.3%), despite the treatment. Mortality was significantly associated with the presence of renal failure with a hazard ratio (HR) of 3.4 (95% CI of 1.2-9.9, p =0.025). Treatment of the underlying disease proved to be protective against mortality with an HR of 0.3 (95% CI 0.1 to 0.98, p = 0.046).Conclusions:
The prognosis of HLH could be related to the treatment of the underlying disease. The study of the pathophysiology of this syndrome will allow a better understanding and treatment.
Full text:
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Index:
LILACS (Americas)
Main subject:
Lymphohistiocytosis, Hemophagocytic
Type of study:
Etiology study
/
Incidence study
/
Observational study
/
Prognostic study
/
Risk factors
Limits:
Adolescent
/
Adult
/
Aged
/
Aged80
/
Female
/
Humans
/
Male
Country/Region as subject:
South America
/
Argentina
Language:
Spanish
Journal:
Rev. méd. Chile
Journal subject:
Medicine
Year:
2017
Type:
Article
Affiliation country:
Argentina
Institution/Affiliation country:
Hospital Italiano de Buenos Aires/AR
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