title: Activity of single-agent decitabine in atypical chronic myeloid leukemia.
abstract: Atypical chronic myeloid leukemia is a rare entity that presents diagnostic and therapeutic challenges. Traditionally utilized therapeutic agents such as hydroxyurea or interferon result in a median survival of approximately two years, thus warranting identification of better options. We report a 49-year-old Caucasian female, who presented with extreme leukocytosis (white blood cells of 148,300/µL) with left shift, severe anemia, and thrombocytopenia. Following a diagnosis of atypical chronic myeloid leukemia, she was started on intravenous decitabine. She subsequently developed paraneoplastic vasculitis of large arteries, which responded to high-dose glucocorticoid. Decitabine therapy resulted in an excellent hematologic response, transfusion independence, and successful transition to an allogeneic peripheral stem cell transplantation. However, the patient subsequently succumbed to the complications of acute graft-versus-host-disease. This case illustrates an association between atypical chronic myeloid leukemia and steroid-responsive paraneoplastic vasculitis and highlights the single-agent disease activity of decitabine in atypical chronic myeloid leukemia, which may be utilized as a bridging therapy to allogeneic stem cell transplantation.
authors.affiliation: Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, USA.
authors.forename: Heidi
authors.initials: H
authors.lastname: Hausmann
authors.affiliation: Division of Hematology and Oncology, Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, USA vijaya.bhatt@unmc.edu.
authors.forename: Vijaya R
authors.initials: VR
authors.lastname: Bhatt
authors.affiliation: Division of Hematology and Oncology, Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, USA.
authors.forename: Ji
authors.initials: J
authors.lastname: Yuan
authors.affiliation: Division of Hematology and Oncology, Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, USA.
authors.forename: Lori J
authors.initials: LJ
authors.lastname: Maness
authors.affiliation: Department of Internal Medicine, Veteran's Affairs Nebraska-Western Iowa Health Care System, Omaha, NE, USA Division of Hematology and Oncology, Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, USA.
authors.forename: Apar K
authors.initials: AK
authors.lastname: Ganti
mesh_terms: D061605:Administration, Intravenous; D000964:Antimetabolites, Antineoplastic / Q000008:administration & dosage*; D001374:Azacitidine / Q000008:administration & dosage / Q000031:analogs & derivatives*; D000077209:Decitabine; D005260:Female; D006086:Graft vs Host Disease / Q000175:diagnosis / Q000517:prevention & control; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D006801:Humans; D054438:Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative / Q000175:diagnosis* / Q000188:drug therapy*; D008875:Middle Aged; D014184:Transplantation, Homologous
chemical_list: D000964:Antimetabolites, Antineoplastic; D000077209:Decitabine; D001374:Azacitidine
keywords: Atypical chronic myeloid leukemia; decitabine; hematopoietic stem cell transplant; paraneoplastic vasculitis