Lang Claudia C V, Ramelyte Egle, Dummer Reinhard
Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
Medical Faculty, University of Zurich, Zurich, Switzerland.
Front Oncol. 2020 Aug 7;10:1163. doi: 10.3389/fonc.2020.01163. eCollection 2020.
Primary cutaneous B-cell lymphomas (pCBCL) include an infrequent group of non-Hodgkin lymphomas that are limited to skin sites at the time of diagnosis. They comprise roughly 20-25% of all cutaneous lymphomas and are subdivided into primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large cell B cell lymphoma, leg type (PCDLCBCL, LT). The first two show a rather indolent course while PCDLCBCL, LT carries a worse prognosis. Intravascular large cell B-cell lymphoma is the most infrequent subtype, and its therapy is not covered in this review. For solitary, single-site PCMZL and PCFCL, several topical treatment options exist. They include, but are not limited to, excision, radiotherapy, and intralesional therapies, discussed in this review. However, in selected cases, even "watchful waiting" is reasonable. Indolent types of pCBCL rarely require systemic treatment. However, in extended cases and more importantly DLCBCL, LT, systemic treatment is the first choice. Monoclonal anti-CD20-antibody rituximab is often used as monotherapy in PCMZL and PCFCL or combined with chemotherapy in PCDLBCL, LT. Newer options are monoclonal anti-CD40 antibody dacetuzumab, anti-PD-1 and anti-PD-L1 checkpoint inhibitors, and Bruton tyrosine kinase inhibitors. Indolent pCBCL are treated with a risk-adapted strategy using intralesional steroids, RT, and interferon-α as first-line treatments. Relapsing cases may profit from rituximab. In aggressive PCDLCBCL, LT, rituximab with polychemotherapy is recommended. Innovative therapies include intralesional oncolytic virotherapy, systemic monoclonal antibodies, and small molecules.
原发性皮肤B细胞淋巴瘤（pCBCL）是一组罕见的非霍奇金淋巴瘤，诊断时局限于皮肤部位。它们约占所有皮肤淋巴瘤的20%-25%，可细分为原发性皮肤边缘区淋巴瘤（PCMZL）、原发性皮肤滤泡中心淋巴瘤（PCFCL）和原发性皮肤弥漫性大细胞B细胞淋巴瘤，腿部型（PCDLCBCL，LT）。前两种表现为相对惰性的病程，而PCDLCBCL，LT的预后较差。血管内大细胞B细胞淋巴瘤是最罕见的亚型，本综述不涉及其治疗。对于孤立的、单部位的PCMZL和PCFCL，有几种局部治疗选择。它们包括但不限于切除、放疗和皮损内治疗，本综述将对此进行讨论。然而，在某些情况下，“观察等待”也是合理的。惰性类型的pCBCL很少需要全身治疗。然而，在病情扩展的情况下，更重要的是在DLCBCL，LT中，全身治疗是首选。单克隆抗CD20抗体利妥昔单抗通常在PCMZL和PCFCL中用作单一疗法，或在PCDLBCL，LT中与化疗联合使用。新的选择包括单克隆抗CD40抗体达西珠单抗、抗PD-1和抗PD-L1检查点抑制剂以及布鲁顿酪氨酸激酶抑制剂。惰性pCBCL采用风险适应性策略治疗，使用皮损内类固醇、放疗和干扰素-α作为一线治疗。复发病例可能从利妥昔单抗中获益。在侵袭性PCDLCBCL，LT中，推荐利妥昔单抗联合多药化疗。创新疗法包括皮损内溶瘤病毒疗法、全身单克隆抗体和小分子药物。
