title: Early relapse of JAK2 V617F-positive chronic neutrophilic leukemia with central nervous system infiltration after unrelated bone marrow transplantation.
abstract: Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative disorder characterized by a proliferation mainly of mature neutrophils. The prognosis is generally poor and an optimal therapeutic strategy remains to be determined. Allogeneic hematopoietic stem cell transplantation (HSCT) is expected to be the only curative therapy so far. We report a 46-year-old male with progressive CNL who underwent bone marrow transplantation from an HLA-matched unrelated donor. After engraftment was achieved on day 35, relapse of CNL was confirmed on day 50. The progression of CNL was very rapid afterward and infiltration to the central nervous system was observed. The Janus Kinase 2 (JAK2) V617F homozygous mutation was detected from the peripheral blood or bone marrow samples throughout the clinical course. From comparison with reports of successful HSCT for CNL in the literature, it was inferred that HSCT should be performed in a stable status before progression. Furthermore, JAK2 V617F-positive CNL may contain an aggressive disease entity in contrast to previous reports. Accumulation of experiences is required to establish a definite role of HSCT in the treatment of CNL and a prognostic significance of JAK2 mutation in CNL.
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: Shinichi
authors.initials: S
authors.lastname: Kako
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Kanda
authors.forename: Tomohiko
authors.initials: T
authors.lastname: Sato
authors.forename: Susumu
authors.initials: S
authors.lastname: Goyama
authors.forename: Naohiro
authors.initials: N
authors.lastname: Noda
authors.forename: Eriko
authors.initials: E
authors.lastname: Shoda
authors.forename: Kumi
authors.initials: K
authors.lastname: Oshima
authors.forename: Morihiro
authors.initials: M
authors.lastname: Inoue
authors.forename: Koji
authors.initials: K
authors.lastname: Izutsu
authors.forename: Takuro
authors.initials: T
authors.lastname: Watanabe
authors.forename: Toru
authors.initials: T
authors.lastname: Motokura
authors.forename: Shigeru
authors.initials: S
authors.lastname: Chiba
authors.forename: Masashi
authors.initials: M
authors.lastname: Fukayama
authors.forename: Mineo
authors.initials: M
authors.lastname: Kurokawa
mesh_terms: D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001088:Arabinonucleotides / Q000008:administration & dosage; D016026:Bone Marrow Transplantation; D001921:Brain / Q000473:pathology*; D007446:Chromosome Inversion; D002899:Chromosomes, Human, Pair 9 / Q000648:ultrastructure; D003131:Combined Modality Therapy; D003568:Cytidine Monophosphate / Q000008:administration & dosage / Q000031:analogs & derivatives; D004401:Dysarthria / Q000209:etiology; D017809:Fatal Outcome; D006801:Humans; D006918:Hydroxyurea / Q000008:administration & dosage / Q000627:therapeutic use; D006941:Hyperesthesia / Q000209:etiology; D053614:Janus Kinase 2 / Q000235:genetics*; D015467:Leukemia, Neutrophilic, Chronic / Q000188:drug therapy / Q000201:enzymology / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D017254:Leukemic Infiltration; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000235:genetics*; D012008:Recurrence; D014184:Transplantation, Homologous
chemical_list: D018906:Antineoplastic Agents, Alkylating; D001088:Arabinonucleotides; D009363:Neoplasm Proteins; C061162:1-arabinofuranosylcytosine-5'-stearylphosphate; C507924:JAK2 protein, human; D053614:Janus Kinase 2; D003568:Cytidine Monophosphate; D006918:Hydroxyurea