Fulminant, CMV-associated, haemophagocytic syndrome following unrelated bone marrow transplantation

We report a case of haemophagocytic syndrome (HPS) occurring after allogene ic bone marrow transplantation (BMT) for acute promyelocytic leukaemia (APL ) in a patient in fourth complete remission (CR), Anticytomegalovirus (CMV) antibody (Ab) was negative in this patient before BMT, BMT was performed f rom an HLA-identical unrelated donor who was positive for CMV Ab, After bon e marrow engraftment and haematological recovery, severe acute graft-versus -host disease (GVHD) developed. This patient was treated with methylprednis olone in addition to cyclosporin A (CsA), Acute GVHD showed partial improve ment, but CMV antigenaemia was observed. Despite administration of gancyclo vir and immunoglobulin, CMV antigenaemia showed no improvement and HPS deve loped. As no other infections or malignancies were observed, we suspect tha t CMV infection was the trigger for development of HPS.