Report of nine cases of aggressive natural killer-cell leukemia / 白血病·淋巴瘤

ABSTRACT

Objective To identify the clinical and pathological features of aggressive natural killercell leukemia (ANKL). Methods 9 cases of ANKL fulfilling the criteria defined by the World Health Organization classification were retrospectively analyzed with literature review, Results Systemic symptoms, hepatomegaly, splenomegaly, lymphadenopathy were frequently observed. Liver dysfunction, neutropenia, anemia and thrombocytopenia were often seen during the course of the disease. Most of the bone marrow shows focal or subtle infiltration by the neoplastic cells. The immunophenotype of cells was characteristic for CD+56, sCD-36, and variable expression of CD2, CD7, CD8 and CD11b. T-cell receptor (TCR) genes rearrangement were in germline configuration. Median survival time was 9 weeks. Conclusion ANKL is an entity of mature cytotoxic NK-cell neoplasms with distinct phenotype and disease presentations. The disease often has a fulminant course with a poor response to chemotherapy and a short survival time. Patients achieving CR showed significantly longer survival time, but the remission did not translate into cure of the disease.