title: Blastic natural killer cell leukemia/lymphoma presenting as overt leukemia.
abstract: Blastic natural killer (NK)-cell leukemia/lymphoma is a neoplasm of NK origin with aggressive behavior. The disease affects mainly elderly people and often presents with skin lesions and overt leukemia. Blastic morphology, an NK-cell immunophenotype, and lack of association with Epstein-Barr virus are the clues for the diagnosis. We report herein, the case of a patient with a blastic NK-cell leukemia/lymphoma with overt leukemia at diagnosis, who achieved a complete response after CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) chemotherapy. However, the patient relapsed a few months later and died due to disease progression. Cases of blastic NK-cell leukemia/ lymphoma previously reported are briefly reviewed.
authors.affiliation: Institute of Hematology and Oncology, Department of Hematology, Instituto de Investigaciones Biomédicas August Pi i Sunyer Hospital Clínic, Barcelona, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Alvarez-Larrán
authors.forename: N
authors.initials: N
authors.lastname: Villamor
authors.forename: J C
authors.initials: JC
authors.lastname: Hernández-Boluda
authors.forename: A
authors.initials: A
authors.lastname: Ferrer
authors.forename: M
authors.initials: M
authors.lastname: Camós
authors.forename: E
authors.initials: E
authors.lastname: Campo
authors.forename: A
authors.initials: A
authors.lastname: López-Guillermo
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D002869:Chromosome Aberrations; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000737:chemistry / Q000378:metabolism / Q000473:pathology*; D007945:Leukemia, Lymphoid / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008223:Lymphoma / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012878:Skin Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D015703:Antigens, CD; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone