Adult T-cell leukemia/lymphoma (ATL) presenting in the skin: Clinical, histological and immunohistochemical features of 52 cases
Adult T-cell leukemia/lymphoma (ATL) presenting in the skin: Clinical, histological and immunohistochemical features of 52 cases
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DOI：
10.1080/02841860802657235
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发表时间：
2009-01-01
期刊：
影响因子：
3.1
通讯作者：
Farre, Lourdes
中科院分区：
文献类型：
--
作者：
Bittencourt, Achilea L.;Barbosa, Helenemarie S.;Farre, Lourdes
背景资料。成人T细胞白血病/淋巴瘤(ATL)是由HTLV-I引起的一种严重疾病。本文描述了52例皮肤受累的ATL的临床病理和免疫组织化学表现，并探讨了中位生存时间(MST)与组织学类型、原发皮肤受累和CD8阳性之间的关系。所有病例均为HTLV-I+和HIV-，并经临床分类。在非典型病例中研究HTLV-I前病毒整合。免疫组织化学方法采用CD3、CD4、CD5、CD7、CD8、CD20、CD25、CD30和CD45RO标记。用Ki-67评价细胞增殖指数。结果。原发性27例，继发性25例。所有不典型病例均表现为单克隆性病毒整合。19例表现为类真菌样病变，2例为间变性大细胞淋巴瘤。15例免疫表型不典型，表达CD8。初次皮肤ATL的MST(48个月)长于继发性皮肤ATL(7个月)，差异有统计学意义，但CD8阳性和阴性患者的MST差异无统计学意义。鉴别原发和继发性皮肤ATL并对其进行组织学分类是很重要的，以便更好地评估预后。两种形式的原发皮肤ATL，原发皮肤阴燃和原发皮肤肿瘤(PCT)，也应该被识别。阴燃型患者生存期较长(58个月)，组织学特征提示预后较好，而PCT型患者生存期较短(20个月)，组织学特征提示预后较差。
Background. Adult T-cell leukemia/lymphoma (ATL) is a severe disease caused by HTLV-I. This paper describes the clinicopathological and immunohistochemical findings of 52 cases of ATL with skin involvement and investigates whether there is any relationship between median survival time (MST) and histological patterns, primary cutaneous involvement and CD8 positivity.Material and methods. All cases were HTLV-I+ and HIV- and were clinically classified. HTLV-I proviral integration was investigated in atypical cases. Immunohistochemistry was performed using CD3, CD4, CD5, CD7, CD8, CD20, CD25, CD30 and CD45RO markers. Ki-67 was used to evaluate the proliferative index. Results. Twenty-seven cases were primary, while 25 were secondary. Monoclonal viral integration was demonstrated in all atypical cases. Patterns resembling mycosis fungoides (MF) were found in 19 cases and anaplastic large-cell lymphoma (ALCL) in two cases. Fifteen cases had an atypical immunophenotype and expressed CD8. Primary cutaneous ATL had a longer MST (48 months) than the secondary cutaneous ATL (7 months) and the difference was statistically significant, but no statistically significant difference was found between the MST of CD8-positive and negative cases.Conclusions. It is important to differentiate between primary and secondary cutaneous ATL and classify the cases histologically in order to better evaluate the prognosis. The two forms of primary cutaneous ATL, primary cutaneous smoldering and primary cutaneous tumoral (PCT), should also be identified. The smoldering type presented a longer survival (58 months) and histological aspects suggestive of better prognosis in contrast to the PCT type that had a shorter survival (20 months) and histological characteristics suggestive of worse outcome.