Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Branch, Padua University School of Medicine, Padova, Italy.
Int J Lab Hematol. 2012 Apr;34(2):117-28. doi: 10.1111/j.1751-553X.2011.01374.x. Epub 2011 Sep 6.
The recently updated World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues, published in 2008, has made great advances in revising the disorders previously included in the pool of natural killer (NK) cell tumors. Although NK cell neoplasms represent a relatively rare group of diseases, accounting for <5% of all lymphoid neoplasms, they include very distinctive conditions both clinically and pathologically. This family of diseases includes the most indolent clinical forms, such as the provisional new entry of chronic lymphoproliferative disorder of NK cells (CLPD-NK) in the WHO classification, as well as one of the most fatal diseases recognized in medical oncology, aggressive NK cell leukemia (ANKL), which is characterized by a prognosis of weeks, or even days. In addition, some disorders previously identified as blastic NK cell lymphoma within the NK cell system have been more properly defined and included in the blastic plasmacytoid dentritic cell neoplasms, although rare cases of bona fide immature NK lymphoid tumors (now classified as NK cell lymphoblastic leukemia/lymphoma) have been reported in the literature. This paper focuses on recent concepts and progress in morphology, pathogenesis, clinicopathological features, treatment approaches, and outcomes of NK cell malignancies.
最近更新的 2008 年版世界卫生组织（WHO）造血和淋巴组织肿瘤分类在修订先前归入自然杀伤（NK）细胞肿瘤池的疾病方面取得了重大进展。虽然 NK 细胞肿瘤是一组相对罕见的疾病，占所有淋巴肿瘤的<5%，但它们在临床和病理上都具有非常独特的表现。这组疾病包括最惰性的临床形式，如在 WHO 分类中新增的慢性 NK 细胞淋巴增殖性疾病（CLPD-NK），以及在肿瘤内科中被认为是最致命的疾病之一侵袭性 NK 细胞白血病（ANKL），其预后为数周，甚至数天。此外，以前在 NK 细胞系统中被确定为母细胞性 NK 细胞淋巴瘤的一些疾病已得到更明确的定义，并归入母细胞性浆细胞样树突细胞肿瘤，尽管文献中也有报道少数真正的不成熟 NK 淋巴样肿瘤（现归类为 NK 细胞淋巴母细胞性白血病/淋巴瘤）的病例。本文重点介绍 NK 细胞恶性肿瘤在形态学、发病机制、临床病理特征、治疗方法和预后方面的最新概念和进展。
