Angiotropic lymphoma: An immunophenotypically and clinically heterogeneous lymphoma
Angiotropic lymphoma: An immunophenotypically and clinically heterogeneous lymphoma
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DOI：
10.1038/modpathol.3880450
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发表时间：
2001-11-01
期刊：
影响因子：
7.5
通讯作者：
Hsi, ED
中科院分区：
文献类型：
--
作者：
Yegappan, S;Coupland, R;Hsi, ED
嗜血管性淋巴瘤（AL）是一种少见的淋巴瘤，临床表现无特异性，死亡率高。虽然没有被修订的欧美类淋巴瘤分类特别认可，但它可能会在即将到来的WHO分类中作为弥漫性大B细胞淋巴瘤的亚型出现。有些作者也可能认为它是皮肤淋巴瘤的一个亚型。最近的研究报告了AL的免疫表型异质性，在罕见的情况下，与其他NHL的关联。为了进一步描述AL的特征，我们通过免疫组化研究了在北美三个医学中心鉴定的18例B细胞AL中的CD 5、CD 10、CD 20、bcl-2和bcl-6的免疫表型。用聚合酶链反应检测Bcl-2基因重排状态，用原位杂交检测Epstein巴尔病毒状态。8名男性和10名女性被确定为AL（中位年龄71岁）。11名患者在生前被诊断，7名在尸检时被诊断。神经系统症状是最常见的表现，见于6例患者。皮肤是最常见的活检部位。所有病例均显示典型的血管内定位;在2例病例中，在一些器官中还观察到轻微的弥漫性大细胞淋巴瘤成分。大多数（89%）的病例表达bcl-2蛋白，22%的病例表达CD 10、bcl-6和CD 5。根据CD 5和CD 10的表达，有三种主要类型：CD 5-，CD 10-（11例），CD 5+，CD 10-（3例）和CD 5-，CD 10+（3例）。尽管有一名患者在AL之前有滤泡中心淋巴瘤，但我们在这些病例中均未检测到bcl-2基因重排。所有病例均为Epstein巴尔病毒阴性。在接受化疗的五名患者中，有两名完全缓解。基于这些发现，我们得出结论，AL的临床和免疫表型异质性，并可能代表一个以上的致病实体。在某些情况下，AL可能先于另一种淋巴组织增生性疾病，这增加了某些AL病例可能代表另一种类型淋巴瘤转化的可能性。AL的皮肤表现是常见的，然而，它似乎是一种系统性淋巴瘤。虽然经常致命，但早期诊断并接受化疗的AL患者可能会获得缓解。
Angiotropic lymphoma (AL) is an uncommon lymphoma often presenting with nonspecific clinical features and having a high mortality rate. Although not specifically recognized by the Revised European-American Classification of Lymphoid Neoplasms, it likely will appear as a subtype of diffuse large B-cell lymphoma in the upcoming WHO classification. Some authors may also consider it to be a subtype of cutaneous lymphomas. Recent studies have reported an immunophenotypic heterogeneity of AL, and in rare instances, an association with other NHL. To further characterize AL, we studied the immunophenotype by immunohistochemistry for CD5, CD10, CD20, bcl-2, and bcl-6 in 18 cases of B-cell AL identified at three medical centers in North America. Bcl-2 gene rearrangement status by polymerase chain reaction and Epstein Barr virus status by in situ hybridization also were evaluated. Eight men and 10 women were identified with AL (median age 71 years). Eleven patients were diagnosed in life and seven were diagnosed at autopsy. Neurologic symptoms were the most common presentation, seen in six patients. Skin was the most commonly biopsied site. All showed classic intravascular localization; in two cases, there was also a minor diffuse large cell lymphoma component observed in some organs. Most (89%) of the cases expressed bcl-2 protein; CD10, bcl-6 and CD5 were each expressed in 22% of cases. Based on CD5 and CD10 expression, three major groups were evident: CD5-, CD10- (11 cases); CD5+, CD10- (3 cases), and CD5-, CD10+ (3 cases). Even though a follicle center lymphoma preceded the AL in one patient, we did not detect bcl-2 gene rearrangement in any of these cases. All cases were negative for Epstein Barr virus. Of the five treated with chemotherapy, two achieved a complete remission. Based on these findings, we conclude that ALs are clinically and immunophenotypically heterogeneous and may represent more than one pathogenetic entity. In some instances AL may be preceded by another lymphoproliferative disorder, raising the possibility that some cases of AL may represent a transformation from another type of lymphoma. Cutaneous manifestations of AL are common; however, it appears to be a systemic lymphoma. Although often fatal, patients with AL who are diagnosed early and treated with chemotherapy may achieve remission.