SAT0516 CLINICAL CHARACTERISTICS AND PROGNOSTIC FACTORS IN PATIENTS WITH SECONDARY HEMOPHAGOCYTIC SYNDROME
Authors: C. A. Egües Dubuc, A. De Diego, P. Cabrera Miranda, N. Alcorta Lorenzo, J. A. Valero Jaimes, J. R. Furundarena Salsamendi, O. Maiz-Alonso, L. M. Lopez Dominguez, E. Uriarte Isacelaya, J. J. Cancio Fanlo, J. Calvo, J. M. Belzunegui Otano
Journal: Annals of the Rheumatic Diseases (2020)

Background: The Hemophagocytic Syndrome (HPS) had a mortality rate between 20% and 90%. The mortality of HPS secondary to autoimmune diseases (AID) is lower than hemato-oncological diseases (HOD). In general, the HOD, thrombocytopenia, age, and a prolongation of prothrombin are considered to be an adverse prognostic factor.(1) Objectives: To describe and identify differences between patients who survived and did not survive to HPS during hospital admission to a tertiary hospital between 2005 and 2019. Methods: This is a retrospective observational study. All patients who met the diagnostic criteria for LHH were included, or who presented haemophagocytic cells in the bone marrow biopsy, or who had diagnosis of HPS in the hospital discharge report.(2) Demographic, clinical, analytical, etiological, underlying disorder and prognosis variables were collected. Continuous variables are described with the mean or median according to the degree of normality. Kruskal Wallis, Fisher test and Mann-Whitney U test were used for the bivariate analysis, and also a multivariate logistic regression analysis was performed. Results: Thirty patients with HPS were included. They were distributed in 5 subgroups (Table 1). Overall mortality was 43.3%, statistically significant higher in the HOD [8 patients (66.7%); p 0.029]. Also, they were divided into 2 groups (survivor vs. non-survivor; Table 2). In the multivariate model the age and INR prolongation were confirmed to be independently associated with the outcome of mortality. Table 1. Etiology of HPS Etiology n = 30 Mortality AID 10 n = 1 Systemic lupus erythematosus 5 1 Adult Still’s Disease 3 No Rheumatoid arthritis 1 No Sclerosing Disease IgG4 1 No HOD 12 n = 8* Non-Hodgkin’s lymphoma 3 1 Myelodysplastic syndrome 3 2 Acute leukemia 3 3 Extranodal NK cell lymphoma 1 1 Multiple Myeloma 1 No Probable lymphoproliferative process 1 1 Infectious diseases 2 n = 1 Pneumocystis carinii in patient with H.I.V. 1 1 Campylobacter yeyuni 1 No Glyoblastoma multiforme with temozolomida 1 n = 0 HPS without defined aetiology 5 3 HIV: Human Immunodeficiency Virus, NK: Natural Killer. *p = 0,029 Table 2. Characteristics and differences between survivor and non-survivor groups Total Non-survivor survivor n 30 13 17 p&lt;0,05 Age 55,5 ±18,3 68 58,2-74,5 40 34-57 0,043 Women 16 53,3% 7 61,5% 9 47,1% 1,00 Comorbidities (≥ 2 ) 5 16,7% 2 15,4% 3 17,6% 1,000 Hospital stay 35,5 20-60,8 29 15,5-39 13 8-17 0,563 Splenomegaly 16 53,3% 7 53,8% 9 52,9% 1,000 Hepatomegaly 10 33,3% 5 38,5% 5 29,4% 0,705 Hb (g/dL ) 7,1 6,4-7,9 710% 6,2-7,8 7,1 6,6-7,8 0,094 Pt (x10 9 /L ) 13 500 5 000-52 500 16 000 11 000-44 000 12 000 5 000-99 000 0,281 Pt ≤ 100 000 25 83,3% 13 100% 12 70,6% 0,052 Leu (x10 9 /L ) 1 250 238-3 153 1 300 150-3 940 1 400 200-3 340 0,457 Neu (x10 9 /L ) 615 0-1 550 1 290 20-3 300 650 0-1 400 0,805 Fb (mg/dL) (n=24 ) 171 111-358 167,00 106-253 169,00 103-451 0,796 Fer (ng/mL) (n=28 ) 15 330 5 434-38 284 29 063 5 728-74 604 13 225 8 287-28 729 0,108 Tg (mmol/L ) 341 226-438 254,00 184-382 471,00 341-604 0,053 GOT (U/L ) 139 77,5-406,3 133,00 101-513 179,00 101-512,5 0,483 GPT (U/L ) 162 46-389 109,00 41-333 199,00 99-298 0,198 INR (n=29 ) 1,5 1,1-1,9 2,1 1,2-3,7 1,5 1,1-1,6 0,028 Hb: Hemoglobin, Pt: platelets, Leu: leukocytes, Neu: neutrophils, Fb: fibrinogen, Fer: ferritin, Tg: triglycerides, GOT: aspartate aminotransferase, GPT: alanine aminotransferase Conclusion: The HOD presented higher mortality. The non-survivor group presented a longer INR prolongation and a higher age at the time of diagnosis. References: [1]Parikh SA. Prog. factors and outcomes of adults with HLH. Mayo Clin Proc. 2014;89:484–492. [2]Henter JI. HLH-2004: Diag. and therapeutic guidelines for HLH.Pediatr Blood Cancer. 2007;48:124. Disclosure of Interests: César Antonio Egües Dubuc: None declared, Andrea De Diego: None declared, Patricia Cabrera Miranda: None declared, Nerea Alcorta Lorenzo: None declared, Jesús Alejandro Valero Jaimes: None declared, Jose Ramon Furundarena Salsamendi: None declared, Olga Maiz-Alonso: None declared, Luis Maria Lopez Dominguez: None declared, Esther Uriarte Isacelaya: None declared, Jorge Jesús Cancio Fanlo: None declared, Jaime Calvo Grant/research support from: Lilly, UCB, Consultant of: Abbvie, Jansen, Celgene, Joaquin Maria Belzunegui Otano: None declared