Cutaneous Lymphomas: An Update. Part 2: B-Cell Lymphomas and Related Conditions
Cutaneous Lymphomas: An Update. Part 2: B-Cell Lymphomas and Related Conditions
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DOI：
10.1097/dad.0b013e318289b20e
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发表时间：
2014-03-01
影响因子：
1.1
通讯作者：
Mitteldorf, Christina
中科院分区：
文献类型：
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作者：
Kempf, Werner;Kazakov, Dmitry V.;Mitteldorf, Christina
原发性皮肤 B 细胞淋巴瘤 (PCBCL) 是原发性皮肤淋巴瘤的第二常见形式，约占所有原发性皮肤淋巴瘤的 25%-30%。两种形式的低度恶性PCBCL，原发性皮肤滤泡中心淋巴瘤（PCFCL）和粘膜相关淋巴组织型原发性皮肤边缘区淋巴瘤（MALT淋巴瘤）（PCMZL）代表了PCBCL的绝大多数，尽管复发率高，但表现出惰性缓慢进展过程和良好的预后。遗传分析表明 PCMZL 不同于其他形式的结外 MALT 淋巴瘤。更常见的 PCMZL 类别转换和非类别转换形式可以区分为两个独特的子集，它们在细胞组成、IgM 表达和非类别转换形式中发现的皮外受累的生物行为方面有所不同。最近，PCMZL 和 PCFCL 的异常临床和组织学形式被描述为粟粒状或聚集性病变，这在诊断上具有挑战性。与 PCMZL 和 PCFCL 相比，原发性皮肤弥漫性大 B 细胞淋巴瘤、腿型和其他罕见形式的大 B 细胞淋巴瘤（如血管内大 B 细胞淋巴瘤）预后不良。有一组新兴的EB病毒（EBV）驱动的B细胞淋巴增殖，包括发生在免疫功能低下患者中的移植后淋巴增殖性疾病和粘膜皮肤溃疡，以及在衰老相关免疫缺陷的情况下发生的老年人与EBV相关的弥漫性大B细胞淋巴瘤。这篇综述报告了最新的发现，扩大了 PCBCL 的临床病理学特征、鉴别诊断方面和发病机制的范围，并讨论了涉及皮肤的 EBV 相关 B 细胞淋巴增殖。
Primary cutaneous B-cell lymphomas (PCBCL) are the second most common form of primary cutaneous lymphomas and account for approximately 25%-30% of all primary cutaneous lymphomas. Both forms of low-grade malignant PCBCL, primary cutaneous follicle center lymphoma (PCFCL) and primary cutaneous marginal zone lymphoma of mucosa-associated lymphoid tissue-type (MALT lymphoma) (PCMZL) represent the vast majority of PCBCL and show an indolent slowly progressive course and an excellent prognosis despite a high recurrence rate. Genetic analysis indicates that PCMZL differ from other forms of extranodal MALT lymphomas. The more common class-switched and the non-class-switched form of PCMZL can be distinguished as two distinctive subsets that differ in the cellular composition, IgM expression, and biological behavior with extracutaneous involvement found in the non-class-switched form. Recently, unusual clinical and histological forms of PCMZL and PCFCL manifesting with miliary or agminated lesions have been described that are diagnostically challenging. In contrast to PCMZL and PCFCL, primary cutaneous diffuse large B-cell lymphoma, leg type, and other rare forms of large B-cell lymphomas such as intravascular large B-cell lymphoma have an unfavorable prognosis. There is an emerging group of Epstein-Barr virus (EBV)-driven B-cell lymphoproliferations including posttransplant lymphoproliferative disorders and mucocutaneous ulcer occurring in immunocompromised patients and EBV-associated diffuse large B-cell lymphoma of the elderly arising in the setting of senescence-linked immunodeficiency. This review reports on recent findings expanding the spectrum of clinicopathological features, differential diagnostic aspects, and the pathogenesis of PCBCL and discusses the group of EBV-associated B-cell lymphoproliferations involving the skin.