Prognostic Factors of Death in a Cohort of 116 Adults with Hemophagocytic Syndrome: Impact of Underlying Disease and Laboratory Parameters

Pilar Brito-Zerón¹, Pedro Moral Moral², Belchin Kostov³, Luis Caminal-Montero⁴, Guadalupe Fraile⁵, Eva Fonseca⁶, Patricia Pérez Guerrero⁷, Angel Robles⁸, Antonio J. Chamorro⁹, María Andrés Calvo¹⁰, José Ramón Larrañaga¹¹, Maria José Forner¹², Mónica Rodriguez Carballeira¹³, Manuel Ruiz Muñoz¹⁴, Roberto Hurtado García¹⁵, Luis Fernando Viejo Llorente¹⁶, Sergio Prieto-González¹⁷, Pedro Castro¹⁸, Aleida Martínez Zapico⁴, María Vaquero Herrero⁹, Angela Ruiz de Temiño de la Peña¹⁰, Soledad Retamozo^1,19, Manuel Ramos-Casals²⁰ and REGHEM-GEAS-SEMI Spanish Cohort, ¹Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Barcelona, Spain, ²Department of Internal Medicine, Hospital La Fé, Valencia, Valencia, Spain, ³Research Group in Primary Care, IDIBAPS, ABS Les Corts, CAPSE, Barcelona, Spain, ⁴Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain, ⁵Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain, Madrid, Spain, ⁶Department of Internal Medicine, Hospital de Cabueñes, Gijón, Gijón, Spain, ⁷Department of Internal Medicine, Hospital Universitario Puerta del Mar, Cádiz, Cadiz, Spain, ⁸Department of Internal Medicine, Hospital La Paz, Madrid, Madrid, Spain, ⁹Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain, ¹⁰Department of Internal Medicine, Hospital Rio Hortega, Valladolid, Valladolid, Spain, ¹¹Department of Internal Medicine, Hospital Xeral, Vigo, Vigo, Spain, ¹²Department of Internal Medicine, Hospital Clínico de Valencia, Valencia, Spain, ¹³Department of Internal Medicine, Hospital Mutua de Terrasa, Terrasa, Spain, ¹⁴Department of Internal Medicine, Hospital Universitario Fundacion Alcorcón, Madrid, Spain, ¹⁵Department of Internal Medicine, Hospital Vega Baja, Orihuela, Orihuela, Spain, ¹⁶Department of Internal Medicine, Hospital Virgen de la Salud, Toledo, Toledo, Spain, ¹⁷Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Barcelona, Spain, ¹⁸Medical Intensive Care Unit, ICMiD, Hospital Clínic, Barcelona, Barcelona, Spain, ¹⁹Rheumatology Unit, Hospital Privado Centro Médico de Córdoba, Argentina, Córdoba, Argentina, ²⁰Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Spain, Barcelona, Spain

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: death and prognostic factors, Disease Activity

Session Information

Date: Tuesday, November 15, 2016

Title: Miscellaneous Rheumatic and Inflammatory Diseases II

Session Type: ACR Concurrent Abstract Session

Session Time: 4:30PM-6:00PM

Background/Purpose: To analyze the potential use of the main features at diagnosis (epidemiological, clinical, laboratory) as prognostic factors and to estimate the risk of death in adult patients with hemophagocytic syndrome (HS).

Methods: In June 2013, the Spanish Autoimmune Diseases Study Group (GEAS-SEMI) created a national registry (REGHEM) of adult patients with HS. Patients were diagnosed according to fulfillment of the criteria of the Histiocytosis Society proposed in 1991 and updated in 2004. The HScore, a prognostic score, which includes 9 clinical, laboratory and histopathological features and ranges from 0 to a maximum of 337 points, was calculated at diagnosis. Time-to-event analyses for death are presented as Kaplan-Meier curves.

Results: By January 2016, the REGHEM registry included 116 adult patients with HS, 68 (59%) men and 48 (41%) women, with a mean age at diagnosis of 49 years (range 14-84 years); 19 (16%) were not born in Spain. The main underlying diseases were chronic infections in 20 (17%) cases, autoimmune/rheumatologic disease in 33 (28%), neoplasia in 23 (22%) and transplantation in 4 (3%); the remaining 36 (31%) patients had no identifiable underlying disease. Sixty-one (53%) patients died. Patients who died were more frequently male (69% vs. 47% in survivors, p=0.03), had a higher mean value of serum ferritin (6662 vs. 3570 ng/mL, p=0.028), a lower mean hemoglobin value (7.6 vs. 8.3 g/dL, p=0.021), a lower mean white blood cell count (1570 vs. 2450 x10⁶/L, p=0.022), a lower mean platelet count (24000 vs. 66000 x10⁶/L, p<0.001), and a higher mean HScore compared with survivors (230 vs. 205, p=0.05). Survival Kaplan-Meier curves (Figure 1) showed that the highest survival rate was in patients with underlying chronic infections while the poorest survival was found in patients with underlying neoplasia (HR 2.41, 95% CI 1.09-5.3).

Conclusion: Hemophagocytic syndrome is a multisystemic disease that remains fatal in > 50% of adults. Survival was significantly reduced in males, and in patients with underlying neoplasia, high ferritin values, severe cytopenias and high HScores. These prognostic factors could help identify patients at greater risk of death, who could benefit from more aggressive specific treatment of potential triggers and intensive supportive care.

Disclosure: P. Brito-Zerón, None; P. Moral Moral, None; B. Kostov, None; L. Caminal-Montero, None; G. Fraile, None; E. Fonseca, None; P. Pérez Guerrero, None; A. Robles, None; A. J. Chamorro, None; M. A. Calvo, None; J. R. Larrañaga, None; M. J. Forner, None; M. Rodriguez Carballeira, None; M. Ruiz Muñoz, None; R. Hurtado García, None; L. F. Viejo Llorente, None; S. Prieto-González, None; P. Castro, None; A. Martínez Zapico, None; M. Vaquero Herrero, None; A. Ruiz de Temiño de la Peña, None; S. Retamozo, None; M. Ramos-Casals, None.

To cite this abstract in AMA style:

Brito-Zerón P, Moral Moral P, Kostov B, Caminal-Montero L, Fraile G, Fonseca E, Pérez Guerrero P, Robles A, Chamorro AJ, Calvo MA, Larrañaga JR, Forner MJ, Rodriguez Carballeira M, Ruiz Muñoz M, Hurtado García R, Viejo Llorente LF, Prieto-González S, Castro P, Martínez Zapico A, Vaquero Herrero M, Ruiz de Temiño de la Peña A, Retamozo S, Ramos-Casals M. Prognostic Factors of Death in a Cohort of 116 Adults with Hemophagocytic Syndrome: Impact of Underlying Disease and Laboratory Parameters [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/prognostic-factors-of-death-in-a-cohort-of-116-adults-with-hemophagocytic-syndrome-impact-of-underlying-disease-and-laboratory-parameters/. Accessed .

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