Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2006
[Aggressive NK-cell leukemia: report of nine cases and review of literature].
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Sino-US Shanghai Leukemia Cooperative Group
Аннотация
OBJECTIVE: To improve the diagnostic accuracy of aggressive NK-cell leukemia (ANKL).
METHODS: Retrospective clinical cases were analysed.
RESULTS AND 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.
METHODS: Retrospective clinical cases were analysed.
RESULTS AND 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.