Author:
Sun Zhe,Wei Ruoqu,Pan Chaolan,Ni Cheng,Zhang Xue,Guan Wenbin,Cheng Ruhong,Gu Yan,Yu Hong,He Kejun,Zhang Zhen,Yu Xia,Yao Zhirong
Abstract
Dedicator of cytokinesis 8 (DOCK8) deficiency represents a primary immunodeficiency with a wide range of clinical symptoms, including recurrent infections, atopy, and increased malignancy risk. This study presents a case of a 6-year-old girl with DOCK8 deficiency, characterized by severe, treatment-resistant herpetic infections who was successfully treated with siltuximab and glucocorticoids. The successful use of siltuximab in achieving remission highlights the pivotal role of interleukin-6 (IL-6) in DOCK8 deficiency pathogenesis and suggests that IL-6 modulation can be critical in managing DOCK8 deficiency-related viral infections, which may inform future therapeutic strategies for DOCK8 deficiency and similar immunodeficiencies.