Ankur Kumar Jindal, Sanjib Mondal, Archan Sil, Amit Rawat, Sanchi Chawla, Rahul Tyagi, Murugan Sudhakar, Aaqib Zaffar Banday, Deepti Suri, Pandiarajan Vignesh, Manpreet Dhaliwal, Saniya Sharma, Rashmi Rikhi, Ruchi Saka, Rajni Sharma, Debajyoti Chatterjee, Sreejesh Sreedharanunni, Ramya Uppuluri, Revathi Raj, Surjit Singh
INTRODUCTION: X-linked lymphoproliferative syndrome (XLP) is a rare primary immune deficiency. Two types of XLP have been described: XLP-1 and XLP-2. METHODS: We found 7 patients with XLP (3 had XLP-1 and 4 had XLP-2) after reviewing the data from Pediatric Immunodeficiency Clinic from 1997 to 2021. RESULTS: Mean age at diagnosis was 3.8 years, and mean delay in diagnosis was 2.6 years. Five patients had recurrent episodes of infections. Four patients developed at least one episode of hemophagocytic lymphohistiocytosis (HLH) (2 with XLP-1 and 2 with XLP-2)...
January 9, 2024: International Archives of Allergy and Immunology