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Pediatric Hemophagocytic Lymphohistiocytosis - A Single Center Study of 118 Patients. OBJECTIVE: To describe the epidemiological features, outcomes and prognostic factors in diagnosis of pediatric hemophagocytic lymphohistiocytosis (HLH). METHODS: Retrospective observational study. 118 children fulfilled the inclusion criteria for HLH between January, 2010 and December, 2019. RESULT: Median age at diagnosis was 4 years (range13 days-15 years). Presenting features were fever (100%), hepatosplenomegaly (91%), neurological symptoms (23%), bicytopenia (76%), transaminitis (67.3%), increased full form (sIL-2r) (78%) and hemophagocytosis on bone marrow (75%). Median serum ferritin was 6504 ng/mL(44-297,000 ng/mL). Median follow up duration was 13.5 months (3 days to 102 months). Primary HLH was identified in 27 (23%) patients. Etiology of secondary HLH was infections 53 (45%), rheumatologic illnesses 21 (18%) and malignancies in 8 (6%) children. Treatment modalities were steroid only (25%), anti-infectious agent (58%), multi-agent chemotherapy (43%) and HSCT (40%); mortality among above treatment groups were 25%, 58%, 43% and 40% respectively. 15 patients (13%) had relapsed/refractory HLH who were treated with salvage chemotherapy and hematopoietic stem cell transplantation (HSCT). Mortality was significantly higher in patients with hyponatremia [OR (95% CI) 3.48 (1.35-8.99); P=0.008] hyperbilirubinemia [OR (95% CI) 2.0455 (0.88-4.75); P=0.002], coagulopathy [OR (95% CI) 2.92 (1.15-7.38); P=0.02] and sIL-2r ≥2400 U/mL [OR (95% CI) 9.05 (1.06-77.5); P=0.03).The overall mortality rate was 39 %; mortality within 30 days in 23%. Estimated overall survival (OS) and event free survival (EFS) at 3 years were 62% and 61%, respectively. CONCLUSION: Pediatric HLH is an aggressive disease with high mortality. Hyponatremia, hyperbilirubinemia, coagulopathy and increased sIL2 receptor level at diagnosis predicts poor outcome.