Su I J, Wang C H, Cheng A L, Chen Y C, Hsieh H C, Chen C J, Tien H F, Huang S S, Hu C Y
Department of Pathology, College of Medicine, National Taiwan University, Taipei, Republic of China.
Cancer. 1988 May 15;61(10):2060-70. doi: 10.1002/1097-0142(19880515)61:10<2060::aid-cncr2820611022>3.0.co;2-d.
Postthymic T-cell malignancy shows marked geographic, clinicopathologic, and prognostic diversity. The frequency and spectrum of T-cell malignancies in Taiwan were investigated. Fifty-two patients (35 male and 17 female) with a median age of 49 years, were consecutively encountered between October 1983 and April 1987; these accounted for 39% of non-Hodgkin's lymphoma cases seen in our institutions. Ten patients (19.3%) had adult T-cell leukemia/lymphoma (ATL) associated with human T-cell leukemia virus (HTLV-1). Patients with ATL had disease similar to that reported from southwestern Japan and the Caribbean. They had frequent skin lesions (60%), hypercalcemia (40%), and a rapid clinical course with a median survival of 1.3 years. The 35 HTLV-1-negative peripheral T-cell lymphomas (PTL) were similar to PTL in western countries, manifesting frequent visceral, cutaneous, and vascular tropisms. Marrow involvement was documented at presentation in 39% and Stage III/IV disease in 80% of the PTL patients. The histology of PTL usually expressed prominent reactive features which is distinct from that in ATL. Several subcategories could be defined: Hodgkin's-like PTL in nine patients, T-zone lymphoma in three, angioimmunoblastic lymphadenopathy-like lymphoma in one, Lennert's lymphoma in three, and angioinvasive lymphoma in four. Two HTLV-1-negative PTL had neoplastic cells with clover-shaped nuclei and were designated as ATL-like. Morphologic classification based on the modified Working Formulation showed prognostic correlation, with median survival of less than 6 months for large cell/immunoblastic PTL, compared with 5 years for patients with small/medium cell PTL. Both low- and high-grade PTL seem to represent an incurable disease. Classical cutaneous T-cell lymphoma (seven cases) is relatively unusual in Taiwan, compared with the frequency of PTL. Post-thymic T-cell malignancies in Taiwan include HTLV-1-positive and HTLV-1-negative diseases, both of which have a poor prognosis and resemble similar T-cell malignancies in the East and West.
胸腺后T细胞恶性肿瘤表现出显著的地域、临床病理及预后多样性。我们对台湾地区T细胞恶性肿瘤的发病率及谱系进行了调查。在1983年10月至1987年4月期间，我们连续收治了52例患者（男性35例，女性17例），中位年龄49岁；这些患者占我们机构所见非霍奇金淋巴瘤病例的39%。10例患者（19.3%）患有与人类T细胞白血病病毒（HTLV-1）相关的成人T细胞白血病/淋巴瘤（ATL）。ATL患者的病情与日本西南部及加勒比地区报道的相似。他们常有皮肤病变（60%）、高钙血症（40%），临床病程进展迅速，中位生存期为1.3年。35例HTLV-1阴性外周T细胞淋巴瘤（PTL）与西方国家的PTL相似，常累及内脏、皮肤和血管。39%的PTL患者初诊时即有骨髓受累，80%的患者为Ⅲ/Ⅳ期疾病。PTL的组织学表现通常具有明显的反应性特征，这与ATL不同。可分为几个亚类：9例霍奇金样PTL、3例T区淋巴瘤、1例血管免疫母细胞性淋巴结病样淋巴瘤、3例 Lennert淋巴瘤和4例血管侵袭性淋巴瘤。2例HTLV-1阴性PTL的肿瘤细胞具有三叶草形核，被归为ATL样。基于改良工作分类法的形态学分类显示出预后相关性，大细胞/免疫母细胞性PTL的中位生存期少于6个月，而小/中细胞性PTL患者的中位生存期为5年。低级别和高级别PTL似乎均为不可治愈的疾病。与PTL的发病率相比，经典皮肤T细胞淋巴瘤（7例）在台湾相对少见。台湾地区的胸腺后T细胞恶性肿瘤包括HTLV-1阳性和HTLV-1阴性疾病，二者预后均较差，且与东西方相似的T细胞恶性肿瘤类似。
