Mature natural killer cell neoplasms: extra nodal NK cell lymphoma and aggressive NK cell leukemia
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摘要: 目的：探讨成熟NK细胞肿瘤的临床特点、诊断及治疗效果。方法：收集14例成熟NK细胞肿瘤病例并进行回顾性分析。结果：侵袭性NK细胞白血病（ANKL）4例，结外NK细胞淋巴瘤鼻型（ENKL）10例。4例ANKL病情呈暴发性，进展快，临床表现为发热，肝脾肿大，贫血，多脏器功能衰竭，均治疗无效死亡。10例ENKL临床特点为局部肿块，溃疡坏死伴出血或脓性分泌物。5例死亡，5例正在治疗中。结论：成熟NK细胞肿瘤较罕见，目前对其发病机制不明，ANKL诊断主要依据临床表现、骨髓细胞学检查和免疫学分型，采用急性淋巴细胞白血病的化疗方案治疗。ENKL诊断主要依据临床表现、组织活检和免疫组化，采用化放疗联合治疗。Abstract: Objective: To explore the clinical characteristics, diagnosis and therapeutic effect of mature natural killer (NK) cell neoplasms in order to enhance the recongnization of the disease.Method: Clinical data of 14 patients with mature NK cell neoplasms were analyzed retrospectively.Result: Mature NK cell neoplasms is consited of aggressive NK cell leukemia (ANKL) and extra nodal NK cell lymphoma nasal type(ENKL).The disease course was fulminant and developed rapidly in 4 cases with aggressive NK cell leukemia (ANKL) which presented with fever, hepatosplenomegaly, anemia and multiple organ failure.The therapeutic effect was poor and the patients died.Ten cases of ENKL mostly occured in the nasal or par nasal area.Five cases died and others were still in follow-up.Conclusion: NK cell neoplasm is extremely rare and its pathogenesis is unknown.The diagnosis of ANKL is based on clinical manifestations, bone marrow cytology and immunophenotyping.Chemotherapy of ANKL is as same as that of acute lymphoblastic leukemia.The diagnosis of ENKL is based on clinical manifestations, tissue biopsy and immunohistochemistry.Treatment of ENKL is chemotherapy and radiotherapy.
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