Clinical Characteristics, Prognostic Factors and Outcomes of Paediatric Patients with Haemophagocytic Lymphohistiocytosis

Abstract

Objective: Haemophagocytic lymphohistiocytosis (HLH) is a multi-system autoimmune disorder. The objective of this study was to find out the clinical characteristics and prognosis of paediatric patients with Haemophagocytic Lymphohistiocytosis. Study Design and setting: Cross sectional study conducted at Pak Emirates Military Hospital, Rawalpindi from July 2021 to June 2023. Methodology: Children with diagnosis of HLH were assessed by including patients who were aged = 13 years during hospitalization. All the patients who had not been diagnosed by using the HLH-2004 criteria were disqualified. Relevant findings were noted by evaluating records pertinent to physical examination, radiology and laboratory markers. Prognosis was assessed by determining the underlying clinical aetiology and whether patient-related factors modulated the overall life expectancy. Results: A total of 32 patient records were evaluated. Mean age at diagnosis was 44.3 ± 39.1 months (Range: 1-132 months) with majority being males [n=23 (71.9%)]. The common clinical characteristics included fever [n=29 (90.6%)], lymphadenopathy [n=27 (84.4%)], splenomegaly [n=23 (71.9%)] and hepatomegaly [n=23 (71.9%)]. Serum ferritin, bilirubin, ALT, AST, and LDH were also raised. All patients were followed for a mean period of 12 months and 18 (56.3%) children failed to survive. Negative prognostic indicators included severe anaemia (p=0.001), neutropenia (p=0.007), thrombocytopenia (p=0.033), and hyperferritinemia (p<0.001). Elevation of liver enzymes (ALT: p<0.001; AST: p=0.031), serum bilirubin (p=0.037), and LDH (p<0.001) also indicated worse disease prognosis. Conclusion: HLH in childhood is a potentially life-threatening disease and carries a significant association with deranged liver function