CLINICAL FEATURES AND TREATMENT OUTCOMES OF LIVER INVOLVEMENT IN PAEDIATRIC LANGERHANS CELL HISTIOCYTOSIS
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Abstract
Introduction: Langerhans cell histiocytosis (LCH) is a rare and heterogeneous disorder characterized by the abnormal proliferation of Langerhans cells, a type of dendritic cell involved in the immune response.
Objectives: The main objective of the study is to find the clinical features and treatment outcomes of liver involvement in paediatric Langerhans cell histiocytosis (LHC).
Methodology of the study: This retrospective observational study was conducted at Bolan Medical College Quetta from 2023 to 2024. The study included 85 pediatric patients diagnosed with LCH who presented with liver involvement. Data were collected from patient medical records, demographic information, age, gender, clinical presentation, symptoms, physical examination findings, and laboratory results, imaging studies, ultrasound, CT, and MRI findings indicative of liver involvement, histopathological findings, biopsy results confirming liver infiltration by Langerhans cells, treatment regimens and follow-up data were also collected.
Results: Data consisted of 85 pediatric patients with Langerhans cell histiocytosis (LCH) and liver involvement, with a mean age at diagnosis of 4.5 years (± 1.8). The gender distribution was fairly balanced, with 46 males (54%) and 39 females (46%). Clinically, hepatomegaly was observed in 72% of patients, while elevated liver enzymes were common, with mean AST, ALT, and ALP levels of 85 U/L (± 15), 78 U/L (± 14), and 260 U/L (± 50), respectively.The overall cohort had an overall survival rate of 85% and an event-free survival rate of 65%. Age at diagnosis showed that patients ≤ 5 years had higher survival rates (88% overall, 68% event-free) compared to those > 5 years (82% overall, 62% event-free).
Conclusion: Pediatric Langerhans cell histiocytosis (LCH) with liver involvement presents significant clinical challenges, marked by hepatomegaly, elevated liver enzymes, and diverse treatment responses.
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Muhammad Qayyum
Consultant Haematologist, Department of Pathology, Bolan Medical College, Quetta
Youksal Khan
House Officer, Oncology Department, Jinnah Postgraduate Medical Centre, Karachi
Mian Muhammad Umer Farooq
General Practitioner, General Medicine Department, Canadian Medical Center, Abu Dhabi
Muhammad Javaid Iqbal
Assistant Professor, Pathology Department, Aziz Fatima Medical and Dental College, Faisalabad
Sarooj Gul
Trainee Medical Officer, Peaditric B Ward, Hayatabad Medical Complex, Peshawar
Aneesh N Jadhav
Paediatric House Officer, Paediatric Department, Dr BR Ambedkar Hospital, Mumbai
Sara Andleeb
Doctor, Pediatrics Medicine Department, Liaquat National Hospital, Pakistan
Misbah Ayub
Resident Medical Officer, Internal Medicine Department, Dow University of Health Sciences, Karachi
Lal Muhammad
Assistant Professor, Pediatric Department, Lady Reading Hospital MTI, Peshawar