Successful reduced-intensity umbilical cord blood transplant for fulminant hemophagocytic syndrome in an adult with pre-existing rheumatoid arthritis and autoimmune hemolytic anemia

摘要

Hemophagocytic syndrome (HPS) is a potentially fatal syndrome of dysregulated immune activation characterized by severe clinical manifestations such as pancytopenia, high fever and hepatosplenomegaly. In adults, it usually occurs in association with a variety of underlying immune abnormalities including lymphoproliferative disorders (LPDs), viral infections and collagen vascular diseases. Most cases of mild to moderate severity HPS respond to high-dose steroid therapy or immunochemotherapy, but the treatment of severe HPS with pharmacologic therapy alone is often difficult. A few previous studies have reported that allogeneic hematopoietic cell transplant (HCT) is an effective therapy for refractory HPS, especially in inherited cases in children [1]. However, the benefit of allogeneic HCT in sporadic HPS in adults has not yet been established [2].