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台湾单家机构中以白血病表现的 T 细胞和自然杀伤细胞/T 细胞肿瘤谱。

The spectrum of T-cell and natural killer/T-cell neoplasms with leukaemic presentation in a single institution in Taiwan.

机构信息

Department of Pathology, Chi-Mei Medical Centre, Tainan, Taiwan.

出版信息

Int J Lab Hematol. 2012 Aug;34(4):422-6. doi: 10.1111/j.1751-553X.2012.01407.x. Epub 2012 Feb 16.

DOI:10.1111/j.1751-553X.2012.01407.x
PMID:22341045
Abstract

T cell and natural killer (NK)/T-cell neoplasms are rare and may occasionally present as leukaemia. We retrospectively searched T cell and NK/T-cell tumours in a single institution in Taiwan from January 2000 to December 2009 and identified 137 (19.1%) patients with T cell and NK/T-cell tumours among 718 patients with lymphoid neoplasms. Among these 137 patients, 18 (13.1%) presented with leukaemia including T-lymphoblastic lymphoma/leukaemia (T-LBL), T-cell prolymphocytic leukaemia, aggressive NK-cell leukaemia, adult T-cell lymphoma/leukaemia (ATLL), T-cell large granular lymphocytic (T-LGL) leukaemia and unspecified peripheral T-cell lymphoma. Cases with concurrent lymphoma, higher absolute leukaemic cell counts and elevated lactate dehydrogenase level carried a poorer prognosis. The survival was dichotomous, with a very poor prognosis for patients with T-LBL, T-cell prolymphocytic leukaemia, aggressive NK-cell leukaemia, ATLL in acute phase and unspecified peripheral T-cell lymphoma, while those with T-LGL leukaemia and ATLL in chronic phase had a favourable outcome.

摘要

T 细胞和自然杀伤(NK)/T 细胞肿瘤较为罕见,偶可表现为白血病。我们回顾性检索了 2000 年 1 月至 2009 年 12 月在台湾一家机构的 T 细胞和 NK/T 细胞肿瘤病例,并在 718 例淋巴肿瘤患者中发现 137 例(19.1%)T 细胞和 NK/T 细胞肿瘤患者。在这 137 例患者中,18 例(13.1%)表现为白血病,包括 T 淋巴母细胞淋巴瘤/白血病(T-LBL)、T 细胞前体淋巴细胞白血病、侵袭性 NK 细胞白血病、成人 T 细胞淋巴瘤/白血病(ATLL)、T 细胞大颗粒淋巴细胞白血病和未特指的外周 T 细胞淋巴瘤。伴有同时性淋巴瘤、更高的绝对白血病细胞计数和升高的乳酸脱氢酶水平的病例预后较差。生存情况呈二项分布,T-LBL、T 细胞前体淋巴细胞白血病、侵袭性 NK 细胞白血病、急性期 ATLL 和未特指的外周 T 细胞淋巴瘤患者的预后极差,而 T-LGL 白血病和慢性期 ATLL 患者的预后较好。

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