AB1473 HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS AMONG ADULTS

Background Hemophagocytic Lymphohistiocytosis (HLH) is a rare life-threatening condition resulting from an overstimulation of the immune system leading to multi-organ damage[1]. This can be either primary caused by genetic mutations or secondary triggered infections, autoimmune diseases malignancies[1]. Objectives We sought examine clinical characteristics, underling etiologies, management and outcome adults patients diagnosed with HLH. Methods performed multi-center retrospective study in departments Internal Medicine Infectious Diseases both Fattouma Bourguiba Taher Sfar University hospitals Tunisia. identified 38 meeting HLH-2004 diagnostic criteria. Results There were 24 women 14 men. The male/female sex ratio was 0.58. mean age years (range 18-80 years). Eight (21%) had auto-immune disease: 6 systemic lupus erythematosus, 1 patient rheumatoid arthritis idiopathic thrombocytopenic purpura. Five (13.5%) malignancy. Among biological variable HLH criteria, most common features fever (100%) followed elevated ferritin level (94.7%) cytopenia (84.2%). Triglyceride 76.3% patients. Hemophgocytosis bone marrow found 35 (92.1%). Elevated lactatdshydrogenase cases. underlying etiologies erythematosus (31%) infections (24%), malignancies (16%) adult still’s disease (13%). In our study, 30 (78.9%) underwent intravenous immunoglobulin regimen over 2 days. Twenty-three (60.5%) received and/or oral steroids. Ethoposide prescribed 4 overall mortality 39.5%. achieved remission relapsing later. Mean cause relapses infections. Patients aged above 65 poorer prognosis ( p =0.013). more likely than other = 0.021). Conclusion uncommon but fatal disease. symptoms are non specific misdiagnosis which associated dismal prognosis. Similarly literature female preponderance study[1]. Systemic commonest population disagrees findings similar studies who malignancy as principal triggers[1,2]. A combination steroids infusions 47.3% proven effective first line treatment [3]. Despite rates remained high populations (39.5%) especially among elderly. References [1]Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014; 383(9927):1503–16. [2]Pandey Y, Atwal D, Konda Bimali Middleton Yarlagadda N, et al. lymphohistiocytosis adults. Proc Bayl Univ Med Cent. 2020; 33(3):326–30. [3]Georgiadou S, Gatselis NK, Stefos Zachou K, Makaritsis Rigopoulou EI, Efficient γ-immunoglobulin infusions. World J Clin Cases. 2019; 7(21):3394–406. Acknowledgements: NIL. Disclosure Interests None Declared.