title: Bone marrow transplantation with a reduced-intensity conditioning regimen in a patient with Wegener granulomatosis and therapy-related leukemia.
abstract: We describe a patient with Wegener granulomatosis (WG) who underwent long-term cyclophosphamide treatment and thereafter developed acute myelogenous leukemia (AML). After the AML was induced into remission, the patient received an allogeneic stem cell transplant (allo-SCT) from his sibling after undergoing a reduced-intensity conditioning regimen. His clinical course shortly after allo-SCT was uneventful. No clinically apparent acute or chronic graft-versus-host disease developed. Repeated analysis of the peripheral blood lymphocytes after transplantation showed complete donor chimerism. The level of proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA) remained undetectable until 4 months after transplantation, when it began to increase. When the level of PR3-ANCA peaked, the patient suddenly presented with fever and joint pain, which later spontaneously resolved in parallel with the declining titer of PR3-ANCA. He is now in remission for both AML and WG at 22 months after transplantation. The patient's clinical course after allo-SCT may provide us with valuable information regarding the establishment of allo-SCT as a therapeutic option for WG.
authors.affiliation: Division of Hematology and Rheumatology, Department of Medicine, Kitano Hospital, The Tazuke Kofukai Medical Research Institute, Osaka, Japan. akunitom@kitano-hp.or.jp
authors.forename: Akane
authors.initials: A
authors.lastname: Kunitomi
authors.forename: Takayuki
authors.initials: T
authors.lastname: Ishikawa
authors.forename: Kenichirou
authors.initials: K
authors.lastname: Tajima
authors.forename: Yoshiteru
authors.initials: Y
authors.lastname: Konaka
authors.forename: Masato
authors.initials: M
authors.lastname: Yagita
mesh_terms: D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects*; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects*; D015470:Leukemia, Myeloid, Acute / Q000139:chemically induced / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D033581:Stem Cell Transplantation; D019172:Transplantation Conditioning; D014184:Transplantation, Homologous
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide