SAT0516 CLINICAL CHARACTERISTICS AND PROGNOSTIC FACTORS IN PATIENTS WITH SECONDARY HEMOPHAGOCYTIC SYNDROME

• Published in: Annals of the rheumatic diseases Vol. 79; no. Suppl 1; p. 1214
• Main Authors: Egües Dubuc, C. A., De Diego, A., Cabrera Miranda, P., Alcorta Lorenzo, N., Valero Jaimes, J. A., Furundarena Salsamendi, J. R., Maiz-Alonso, O., Lopez Dominguez, L. M., Uriarte Isacelaya, E., Cancio Fanlo, J. J., Calvo, J., Belzunegui Otano, J. M.
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group LTD 01.06.2020
• Subjects: Age; Alanine; Alanine transaminase; Aspartate aminotransferase; Autoimmune diseases; Biopsy; Bone marrow; Diagnosis; Etiology; Ferritin; Fibrinogen; Hemoglobin; Hemophagocytic syndrome; Immunoglobulin G; Infectious diseases; Leukocytes (neutrophilic); Lymphocytes; Lymphoma; Medical prognosis; Mortality; Multiple myeloma; Myelodysplastic syndrome; Natural killer cells; Non-Hodgkin's lymphoma; Patients; Rheumatoid arthritis; Statistical analysis; Systemic lupus erythematosus
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/annrheumdis-2020-eular.5855

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 HPSEtiologyn = 30MortalityAID10n = 1 Systemic lupus erythematosus51 Adult Still’s Disease3No Rheumatoid arthritis1No Sclerosing Disease IgG41NoHOD12n = 8* Non-Hodgkin’s lymphoma31 Myelodysplastic syndrome32 Acute leukemia33 Extranodal NK cell lymphoma11 Multiple Myeloma1No Probable lymphoproliferative process11Infectious diseases2n = 1 Pneumocystis carinii in patient with H.I.V.11 Campylobacter yeyuni1NoGlyoblastoma multiforme with temozolomida1n = 0HPS without defined aetiology53HIV: Human Immunodeficiency Virus, NK: Natural Killer. *p = 0,029Table 2.Characteristics and differences between survivor and non-survivor groupsTotalNon-survivorsurvivorn301317p<0,05Age55,5±18,36858,2-74,54034-570,043Women1653,3%761,5%947,1%1,00Comorbidities (≥ 2)516,7%215,4%317,6%1,000Hospital stay35,520-60,82915,5-39138-170,563Splenomegaly1653,3%753,8%952,9%1,000Hepatomegaly1033,3%538,5%529,4%0,705Hb (g/dL)7,16,4-7,9710%6,2-7,87,16,6-7,80,094Pt (x109/L)13 5005 000-52 50016 00011 000-44 00012 0005 000-99 0000,281Pt ≤ 100 0002583,3%13100%1270,6%0,052Leu (x109/L)1 250238-3 1531 300150-3 9401 400200-3 3400,457Neu (x109/L)6150-1 5501 29020-3 3006500-1 4000,805Fb (mg/dL) (n=24)171111-358167,00106-253169,00103-4510,796Fer (ng/mL) (n=28)15 3305 434-38 28429 0635 728-74 60413 2258 287-28 7290,108Tg (mmol/L)341226-438254,00184-382471,00341-6040,053GOT (U/L)13977,5-406,3133,00101-513179,00101-512,50,483GPT (U/L)16246-389109,0041-333199,0099-2980,198INR (n=29)1,51,1-1,92,11,2-3,71,51,1-1,60,028Hb: Hemoglobin, Pt: platelets, Leu: leukocytes, Neu: neutrophils, Fb: fibrinogen, Fer: ferritin, Tg: triglycerides, GOT: aspartate aminotransferase, GPT: alanine aminotransferaseConclusion: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