X-Linked Inhibitor of Apoptosis (XIAP) deficiency presenting as hemophagocytic lymphohistiocytosis triggered by drug allergy
X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency associated with recurrent hemophagocytic lymphohistiocytosis (HLH) episodes.The clinical phenotypes of XIAP deficiency vary, ranging from splenomegaly to life-threatening inflammation.We report a case of XIAP deficiency with unusual late-onset HLH presentation likely tr