[Aggressive NK-cell leukemia: report of nine cases and review of literature].
OBJECTIVE
To improve the diagnostic accuracy of aggressive NK-cell leukemia (ANKL).


METHODS
Retrospective clinical cases were analysed.


CONCLUSIONS
The ANKL patients often present with fever, hepatosplenomegaly, jaundice, abnormal liver function and pancytopenia. The disease had an aggressive clinical course. Multiorgan failure and hemophagocytic syndrome were frequent complications. Median survival time was less than 2 months. The diagnostic criteria should be (1) Patients present with fever, hepatosplenomegaly and lymphadenopathy; (2) Neutropenia, anemia and thrombocytopenia with high number of circulating large granular lymphocytes; (3) large granular lymphocytes infiltrated in bone marrow aspirate and core biopsy; (4) immunophenotype: CD2(+), surface CD3(-), cytoplasm CD3(+), CD56(+), CD57(-). CD11b(+/-) and CD16(+/-). No T-cell receptor (TCR) genes rearrangement; (5) EB virus antibody usually positive; (6) No unique karyotypic abnormality, sometimes del (6) (q21q25); (7) Exclusion of other diseases with large granular lymphocytosis.
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D054066:Leukemia, Large Granular Lymphocytic / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies