Aggressive natural killer (NK) cell leukemia (ANKL) is a systemic neoplastic proliferation
of NK cells with an aggressive clinical course. Currently, the diagnosis of ANKL remains
challenging. In the current study, we report the clinical, laboratory, immunophenotypic,
and genetic findings from 29 cases of de novo ANKL in a single center and evaluate the relative contribution of these features
to the diagnosis of ANKL. Clinical features, laboratory findings, morphologic, cytogenetic
features, and Epstein-Barr virus status were important factors for diagnosing aggressive
NK cell leukemia. On the other hand, ANKL displays a strikingly abnormal immunophenotype
in contrast to nonneoplastic NK cells. The immunophenotype of ANKL cells may differ
from reactive NK cells in 4 respects. First, the CD45/linear side scatter gating of
flow cytometry allows the initial identification of neoplastic subpopulations for
additional immunophenotypic analysis in half of ANKL cases. Second, unusual expression
of surface antigens in ANKL cells was a prominent feature. Third, the clonality of
ANKL cells could be identified using antibodies against CD158a/h, CD158b, or CD158e.
Last, the positive rate of Ki-67 expression in ANKL cells was generally high. Based
on these findings, we provide an objective marker based on clinical data for the definite
diagnosis of ANKL.
Abbreviations:
ANKL (aggressive natural killer cell leukemia), AspaMetDex (L-asparaginase, high-dose of methotrexate and dexamethasone), BM (bone marrow), CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), CLPD-NK (chronic lymphoproliferative disorders of natural killer cells), CT (computed tomography), EBV (Epstein-Barr virus), EBER (Epstein-Barr virus-encoded RNA), ENKTL (extranodal natural killer/T-cell lymphoma, nasal type), FDG (fluorodeoxyglucose), IPI (International Prognostic Index), KIR (killer cell immunoglobulin receptor), LDH (lactate dehydrogenase), LGL (large granular lymphocyte), LMP1 (Latent membrane protein 1), NK (natural killer), PB (peripheral blood), PET (positron emission tomography), PCR (polymerase chain reaction), SSC (linear side scatter), TCR (T-cell receptor)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: January 24, 2014
Accepted:
January 16,
2014
Received in revised form:
December 28,
2013
Received:
September 13,
2013
Identification
Copyright
© 2014 Mosby, Inc. Published by Elsevier Inc. All rights reserved.
