Intravascular large T-cell or NK-cell lymphoma - A rare variant of intravascular large cell lymphoma with frequent cytotoxic phenotype and association with Epstein-Barr virus infection
Intravascular large T-cell or NK-cell lymphoma - A rare variant of intravascular large cell lymphoma with frequent cytotoxic phenotype and association with Epstein-Barr virus infection
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DOI：
10.1097/pas.0b013e31815d29c9
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发表时间：
2008-06-01
影响因子：
5.6
通讯作者：
Kerl, Helmut
中科院分区：
文献类型：
--
作者：
Cerroni, Lorenzo;Massone, Cesare;Kerl, Helmut
大多数血管内大细胞淋巴瘤病例具有B细胞表型，但罕见的T细胞和自然杀伤（NK）细胞变异已被报道。我们描述了4例血管内大NK/T细胞淋巴瘤患者（男：女= 3：1;年龄范围：63至87岁;中位年龄：65岁）的临床病理特征。皮肤是所有患者的表现部位（腿部：1例;躯干：1例;躯干和四肢：2例）。2例患者的病变局限于皮肤; 1例检测到脑部伴随受累，1例未进行进一步研究。免疫组化显示3/4例细胞毒性标志物阳性。1例患者的NK表型与鼻型NK/T细胞淋巴瘤相似，而其他病例无法准确分类为特定类别（外周T细胞淋巴瘤，NOS）。其中1例细胞毒性标志物阴性，仅CD 2和CD 3+阳性。原位杂交证实2例与EB病毒（EBV）相关，1例与EBV相关。我们所有的患者都有侵袭性疾病，并在发病后2周至7个月内死亡。对我们的病例和文献中发表的病例的分析表明，血管内大NK/T细胞淋巴瘤是一种罕见的侵袭性淋巴瘤，具有可变的表型特征，细胞毒性蛋白的频繁表达，真正的NK细胞表型，与EB病毒感染相关，常见于皮肤。需要对大量患者进行同质性研究，并重新评估已发表的表型数据不完整的病例，以收集有关这种极其罕见的淋巴瘤类型的更多信息。
Most cases of intravascular large cell lymphoma have a B-cell phenotype, but rare T-cell and natural killer (NK)-cell variants have been reported. We describe the clinicopathologic features of 4 patients (M:F = 3:1; age range: 63 to 87; median age: 65) with intravascular large NK/T-cell lymphoma. The skin was the site of presentation in all patients (leg: 1 case; trunk: 1 case; trunk and extremities: 2 cases). Two patients had lesions confined to the skin; in 1 case concomitant involvement of the brain was detected and in 1 case no further studies were carried out. Immunohistology showed positivity for cytotoxic markers in 3/4 cases. One case had an NK phenotype similar to NK/ T-cell lymphoma, nasal-type, whereas the other cases could not be precisely classified into specific categories (peripheral T-cell lymphoma, NOS). One of these cases was negative for cytotoxic markers and was positive only for CD2 and CD3 epsilon. Association with Epstein-Barr virus (EBV) was demonstrated in 2 cases by in situ hybridization, whereas I case was negative. All our patients had aggressive disease and died between 2 weeks and 7 months from presentation. Analysis of our cases and of those published in the literature shows that intravascular large NK/T-cell lymphoma is a rare, aggressive lymphoma with variable phenotypic features, frequent expression of cytotoxic proteins, true NK-cell phenotype and association with Epstein-Barr virus infection, and common presentation in the skin. Homogeneous studies on larger number of patients and reevaluation of cases published with incomplete phenotypic data would be necessary to gather more information on this extremely rare type of lymphoma.