title: Fulminant, CMV-associated, haemophagocytic syndrome following unrelated bone marrow transplantation.
abstract: We report a case of haemophagocytic syndrome (HPS) occurring after allogeneic bone marrow transplantation (BMT) for acute promyelocytic leukaemia (APL) in a patient in fourth complete remission (CR). Anti-cytomegalovirus (CMV) antibody (Ab) was negative in this patient before BMT. BMT was performed from an HLA-identical unrelated donor who was positive for CMV Ab. After bone marrow engraftment and haematological recovery, severe acute graft-versus-host disease (GVHD) developed. This patient was treated with methylprednisolone in addition to cyclosporin A (CsA). Acute GVHD showed partial improvement, but CMV antigenaemia was observed. Despite administration of gancyclovir and immunoglobulin, CMV antigenaemia showed no improvement and HPS developed. As no other infections or malignancies were observed, we suspect that CMV infection was the trigger for development of HPS.
authors.affiliation: Third Department of Internal Medicine, Gunma University School of Medicine, Maebashi, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Sato
authors.forename: T
authors.initials: T
authors.lastname: Matsushima
authors.forename: S
authors.initials: S
authors.lastname: Takada
authors.forename: N
authors.initials: N
authors.lastname: Hatsumi
authors.forename: K
authors.initials: K
authors.lastname: Kim
authors.forename: M
authors.initials: M
authors.lastname: Sakuraya
authors.forename: T
authors.initials: T
authors.lastname: Saito
authors.forename: J
authors.initials: J
authors.lastname: Tamura
authors.forename: M
authors.initials: M
authors.lastname: Karasawa
authors.forename: H
authors.initials: H
authors.lastname: Murakami
authors.forename: T
authors.initials: T
authors.lastname: Naruse
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000009:adverse effects / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D016572:Cyclosporine / Q000009:adverse effects / Q000627:therapeutic use; D003586:Cytomegalovirus Infections / Q000188:drug therapy / Q000209:etiology; D005260:Female; D006086:Graft vs Host Disease / Q000188:drug therapy / Q000209:etiology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology*; D006650:Histocompatibility Testing; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D015473:Leukemia, Promyelocytic, Acute / Q000628:therapy*; D008775:Methylprednisolone / Q000009:adverse effects / Q000627:therapeutic use; D014184:Transplantation, Homologous
chemical_list: D000893:Anti-Inflammatory Agents; D000998:Antiviral Agents; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D008775:Methylprednisolone