title: Extramedullary blast crisis in a patient with Philadelphia chromosome-positive chronic myelogenous leukemia in complete cytogenetic remission.
abstract: Treatment of Philadelphia (Ph) chromosome-positive chronic myelogenous leukemia (CML) with recombinant interferon-alpha (IFN-A) results in complete disappearance of the Ph chromosome in about 10% to 15% of patients in early chronic phase. This group has a long survival and very low incidence of blast crisis. The first known case is reported of extramedullary blastic transformation in a patient with medullary complete cytogenetic response (0% Ph-positive metaphases) to IFN-A. Four episodes of extramedullary blast crisis have occurred in this patient. The first three episodes were lymphoid by morphology and cytochemical stains. Molecular analysis confirmed breakpoint cluster region rearrangement. The most recent transformation was myeloid in nature and involved bone and pulmonary parenchyma. The patient is currently undergoing a second autologous transplantation with stored bone marrow that is Ph negative. The patient has survived more than 18 months since the first episode of blast crisis, and the bone marrow is normal.
authors.affiliation: Department of Hematology, University of Texas M. D. Anderson Cancer Center, Houston.
authors.forename: S
authors.initials: S
authors.lastname: O'Brien
authors.forename: R
authors.initials: R
authors.lastname: Witt
authors.forename: M
authors.initials: M
authors.lastname: Talpaz
authors.forename: H
authors.initials: H
authors.lastname: Kantarjian
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001752:Blast Crisis / Q000000981:diagnostic imaging / Q000628:therapy*; D016026:Bone Marrow Transplantation; D002908:Chronic Disease; D003131:Combined Modality Therapy; D006801:Humans; D007370:Interferon Type I / Q000627:therapeutic use; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000000981:diagnostic imaging / Q000628:therapy*; D008297:Male; D011877:Radionuclide Imaging; D012074:Remission Induction; D014916:Whole-Body Irradiation
chemical_list: D007370:Interferon Type I