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Brito-Zerón P, Kostov B, Moral-Moral P, et al. Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis. Mayo Clin Proc Innov Qual Outcomes. 2018;2(3):267-276doi: 10.1016/j.mayocpiqo.2018.06.006.
Brito-Zerón, P., Kostov, B., Moral-Moral, P., Martínez-Zapico, A., Díaz-Pedroche, C., Fraile, G., Pérez-Guerrero, P., Fonseca, E., Robles, A., Vaquero-Herrero, M. P., Calvo, M. A., Forner, M. J., Morcillo, C., Larrañaga, J., Rodriguez-Carballeira, M., Ruiz-Muñoz, M., Hurtado-García, R., Prieto-González, S., Aguilar, A. A., Caminal-Montero, L., Hernández-Jiménez, P., Fernández-Viagas, C. R., Castro, P., Massó, V. M., Flores-Chavez, A., Ramos-Casals, M. (2018). Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis. Mayo Clinic proceedings. Innovations, quality & outcomes, 2(3), 267-276. https://doi.org/10.1016/j.mayocpiqo.2018.06.006
Brito-Zerón, Pilar, et al. "Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis." Mayo Clinic proceedings. Innovations, quality & outcomes vol. 2,3 (2018): 267-276. doi: https://doi.org/10.1016/j.mayocpiqo.2018.06.006
Brito-Zerón P, Kostov B, Moral-Moral P, Martínez-Zapico A, Díaz-Pedroche C, Fraile G, Pérez-Guerrero P, Fonseca E, Robles A, Vaquero-Herrero MP, Calvo MA, Forner MJ, Morcillo C, Larrañaga J, Rodriguez-Carballeira M, Ruiz-Muñoz M, Hurtado-García R, Prieto-González S, Aguilar AA, Caminal-Montero L, Hernández-Jiménez P, Fernández-Viagas CR, Castro P, Massó VM, Flores-Chavez A, Ramos-Casals M. Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis. Mayo Clin Proc Innov Qual Outcomes. 2018 Aug 01;2(3):267-276. doi: 10.1016/j.mayocpiqo.2018.06.006. eCollection 2018 Sep. PMID: 30225460; PMCID: PMC6132215.
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Mayo Clin Proc Innov Qual Outcomes. 2018 Aug 01;2(3):267-276. doi: 10.1016/j.mayocpiqo.2018.06.006. eCollection 2018 Sep.

Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis.

Mayo Clinic proceedings. Innovations, quality & outcomes

Pilar Brito-Zerón, Belchin Kostov, Pedro Moral-Moral, Aleida Martínez-Zapico, Carmen Díaz-Pedroche, Guadalupe Fraile, Patricia Pérez-Guerrero, Eva Fonseca, Angel Robles, María P Vaquero-Herrero, María Andrés Calvo, María José Forner, Cesar Morcillo, José Larrañaga, Monica Rodriguez-Carballeira, Manuel Ruiz-Muñoz, Robert Hurtado-García, Sergio Prieto-González, Asun Aljibe Aguilar, Luis Caminal-Montero, Pilar Hernández-Jiménez, Cristina Rodríguez Fernández-Viagas, Pedro Castro, Victoria Morell Massó, Alejandra Flores-Chavez, Manuel Ramos-Casals,

Affiliations

  1. Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA-Sanitas, Barcelona, Spain.
  2. Laboratory of Autoimmune Diseases Josep Font, CELLEX-IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain.
  3. Primary Healthcare Transversal Research Group, IDIBAPS, Barcelona, Spain.
  4. Immunopathology Section, Department of Internal Medicine, Hospital Universitari i Politècnic La Fe, Departament de Salut Valencia La Fe, Valencia, Spain.
  5. Autoimmune Diseases Unit, UGC Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain.
  6. Department of Internal Medicine, Hospital Universitario 12 de Octubre, Madrid, Spain.
  7. Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain.
  8. UGC Internal Medicine Hospital Puerta del Mar de Cádiz, Cádiz, Spain.
  9. Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain.
  10. Department of Internal Medicine, Hospital La Paz, Madrid, Spain.
  11. Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain.
  12. Department of Internal Medicine, Hospital Rio Hortega Valladolid, Valladolid, Spain.
  13. Department of Internal Medicine, Hospital Clínico de Valencia, Valencia, Spain.
  14. Department of Internal Medicine, Hospital Xeral, Vigo, Spain.
  15. Department of Internal Medicine, Hospital Mutua de Terrasa, Terrassa, Spain.
  16. Department of Internal Medicine, Hospital Universitario Fundacion Alcorcón, Alcorcón, Spain.
  17. Department of Internal Medicine, Hospital Vega Baja, Orihuela, Spain.
  18. Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain.
  19. Medical Intensive Care Unit, Department of Medicine, ICMiD, Hospital Clínic, Barcelona, Spain.
  20. Biomedical Research Unit 02, Clinical Epidemiology Research Unit, UMAE, Specialties Hospital, Western Medical Center, Mexican Institute for Social Security (IMSS), Guadalajara, Mexico.
  21. Postgraduate Program of Medical Science, University Center for Biomedical Research (CUIB), University of Colima, Colima, Mexico.
  22. Department of Medicine, University of Barcelona, Barcelona, Spain.

PMID: 30225460 PMCID: PMC6132215 DOI: 10.1016/j.mayocpiqo.2018.06.006

Abstract

OBJECTIVE: To characterize the etiologies and clinical features at diagnosis of patients with hemophagocytic lymphohistiocytosis (HLH) and correlate these baseline features with survival using an etiopathogenically guided multivariable model.

PATIENTS AND METHODS: The Spanish Group of Autoimmune Diseases HLH Study Group, formed in 2013, is aimed at collecting adult patients with HLH diagnosed in internal medicine departments between January 3, 2013, and October 28, 2017.

RESULTS: The cohort consisted of 151 patients (91 men; mean age, 51.4 years). After a mean follow-up of 17 months (range, 1-142 months), 80 patients died. Time-to-event analyses for death identified a worse survival curve for patients with neoplasia (

CONCLUSION: The mortality rate of adult patients diagnosed with HLH exceeds 50%. Infection with more than 1 microbiological agent was the only independent variable associated with mortality irrespective of the underlying disease, epidemiological profile, clinical presentation, and therapeutic management.

Keywords: GC, glucocorticoid; HLH, hemophagocytic lymphohistiocytosis; ICU, intensive care unit; ID, immunosuppressive drug; IVIG, intravenous immunoglobulin; NK, natural killer

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