Hemophagocytic Lymphohistiocytosis Triggered by Legionella pneumophila and SARS-CoV-2 Infection in GATA2 Deficiency.

Germline GATA2 deficiency predisposes to bone marrow failure, myeloid neoplasia, and immune dysregulation. The syndrome is often complicated by infection with intracellular pathogens and viruses, autoimmunity, and inflammation. Hemophagocytic lymphohistiocytosis (HLH) is a rare occurrence that can present further management challenges. Here, we describe a young adult with GATA2 deficiency presenting with Legionella pneumonia, COVID-19, and HLH with underlying SF3B1-mutated myelodysplasia that responded successfully to allogeneic hematopoietic stem cell transplantation.