Primary mediastinal large B-cell lymphoma (PMBCL) is an aggressive B-cell lymphoma originating from the thymus, which has different clinical and biological characteristics from diffuse large B-cell lymphoma, NOS. PMBCL tends to occur in young women, usually presenting as a large anterior mediastinal mass. Most patients are in stage Ⅰ-Ⅱ at the time of presentation. There is no standard prognostic scoring system for PMBCL. Immunochemotherapy is commonly used in the treatment of PMBCL, but the optimal first-line treatment has not been determined, and the status of radiotherapy is controversial. The value of PET-CT guided therapy needs to be further verified. Relapsed/refractory PMBCL has a poor prognosis, while novel therapies such as PD-1 inhibitors, brentuximab vedotin, and CAR-T can help improve survival in these patients.
原发纵隔大B细胞淋巴瘤（PMBCL）是一种起源于胸腺的侵袭性B细胞淋巴瘤。具有不同于非特指型弥漫大B细胞淋巴瘤的临床和生物学特征，PMBCL好发于年轻女性，通常表现为前纵隔巨大肿块，大多数患者就诊时处于Ⅰ～Ⅱ期，目前尚无标准的PMBCL预后评分系统。PMBCL治疗多采用免疫化疗，但最佳一线治疗方案尚无定论，并且放疗的地位存在争议，PET-CT指导治疗的价值有待进一步证实。复发/难治PMBCL预后较差，PD-1抑制剂、维布妥昔单抗和CAR-T细胞等新型疗法有助于改善此类患者生存。.