title: [Clinicopathologic features of aggressive natural killer cell leukemia].
abstract: OBJECTIVE
  To study the clinicopathologic features of aggressive natural killer cell leukemia (ANKL).
  
  
  METHODS
  The clinical and pathologic features were analyzed in 10 patients with ANKL. The complete blood count, peripheral blood smears, bone marrow aspirates and bone marrow biopsies were studied. Immunophenotypic analysis was carried out by flow cytometry and immunohistochemistry. T-cell receptor (TCR) γ gene rearrangement was studied by PCR method.
  
  
  RESULTS
  The most frequent hematologic abnormalities observed were anemia (7 cases) and thrombocytopenia (9 cases). Large granular lymphocytes were found on peripheral blood smears of 6 patients. In bone marrow aspirates, lymphocytosis (> 20.0%) was demonstrated in 8 cases and large granular lymphocytes in 6 cases. Bone marrow biopsies revealed various degrees of neoplastic infiltration, as follows: mild (5 cases), moderate (3 cases) and severe (2 cases). The neoplastic cells were mainly interstitial in distribution in 8 cases and diffuse in 2 cases. Hemophagocytosis was observed in 4 cases. Flow cytometry showed CD2+ sCD3- CD4- CD56+ CD57- in all cases, CD7+ in 9 cases, CD16+ in 5 cases, CD8+ in 4 cases and CD5+ in 1 case. Immunohistochemistry performed in 8 cases showed the following results: cCD3+ in 4 cases, CD56+ in 6 cases, TIA-1+ in 6 cases, granzyme B+ in 4 cases and perforin+ in 2 cases. PCR study revealed germline TCRγ gene configuration in all cases.
  
  
  CONCLUSIONS
  ANKL is a highly aggressive NK cell-derived lymphoid neoplasm. Comprehensive morphologic, immunophenotypic and molecular analysis are essential in arriving at a correct diagnosis. ANKL needs to be distinguished from other types of NK-cell and T-cell lymphomas.
authors.affiliation: Department of Pathology, Chinese Academy of Medical Sciences, Tianjin, China.
authors.forename: En-bin
authors.initials: EB
authors.lastname: Liu
authors.forename: Hui-shu
authors.initials: HS
authors.lastname: Chen
authors.forename: Pei-hong
authors.initials: PH
authors.lastname: Zhang
authors.forename: Zhan-qi
authors.initials: ZQ
authors.lastname: Li
authors.forename: Qi
authors.initials: Q
authors.lastname: Sun
authors.forename: Qing-ying
authors.initials: QY
authors.lastname: Yang
authors.forename: Li-huan
authors.initials: LH
authors.lastname: Fang
authors.forename: Fu-jun
authors.initials: FJ
authors.lastname: Sun
mesh_terms: D000293:Adolescent; D000328:Adult; D001853:Bone Marrow / Q000473:pathology*; D017252:CD3 Complex / Q000378:metabolism; D019002:CD56 Antigen / Q000378:metabolism; D002648:Child; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D016130:Immunophenotyping; D054066:Leukemia, Large Granular Lymphocytic / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D008218:Lymphocytosis; D008297:Male; D008875:Middle Aged; D039102:Poly(A)-Binding Proteins / Q000378:metabolism; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate; D000074019:T-Cell Intracellular Antigen-1; D055815:Young Adult
chemical_list: D017252:CD3 Complex; D019002:CD56 Antigen; D039102:Poly(A)-Binding Proteins; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human