Department of Haematology, Blood Neoplasms, and Bone Marrow Transplantation, Wrocław Medical University, Wroclaw, Poland.
Service d'Hématologie, Centre Hospitalier Le Mans, Le Mans, France.
Acta Oncol. 2021 Oct;60(10):1361-1368. doi: 10.1080/0284186X.2021.1956689. Epub 2021 Aug 4.
Primary cutaneous indolent B-cell lymphomas (PCBCLs) are not well characterized due to their rarity and indolent character. We retrospectively reviewed the data from 52 patients with primary cutaneous follicular lymphoma (PCFL) ( = 26), marginal zone lymphoma (PCMZL) ( = 25) or undefined PCBCL ( = 1) treated in 10 hematology centers in 1999-2019. In almost half of the patients, pruritus or pain were present at diagnosis. The lesions were predominantly located on the head and trunk. The disease was present in a form of solitary infiltration or disseminated lesions with a similar frequency. Surgery, radiotherapy, rituximab alone or combined with chemotherapy were applied as first-line treatment in 33%, 25%, 21% and 21% of patients, with complete response (CR) achieved by 94%, 83%, 50% and 70% of patients, respectively ( = 0.28). The median duration of response (DoR) was 65 months (95%CI 35-155). After the median follow-up time of 46 months (range: 3-225), the estimated 5-year overall survival (OS) and progression-free survival (PFS) were 93% and 54%, respectively. Clinical presentation was largely consistent with the literature data, however, we observed some differences, including higher predilection to affect upper extremities (25%) and more frequent multifocal appearance in PCFCL (64%) and unifocal in PCMZL (70%).A high proportion of patients with indolent PCBCL achieved CR after the first-line therapy (77%), regardless of treatment mode. We did not find any impact of clinical features on treatment outcomes. All treatment modalities resulted in a high overall response rate. Surgery and/or radiotherapy are the optimal therapeutic options for patients with localized disease. The decision to treat systemically should rather be limited to the generalized form of the disease. High response rate, long duration of remission and excellent long-term survival confirm the truly indolent character of PCFCL and PCMZL.
原发性皮肤惰性 B 细胞淋巴瘤（PCBCL）由于其罕见性和惰性特征，尚未得到很好的描述。我们回顾性分析了 1999 年至 2019 年期间 10 个血液学中心治疗的 52 例原发性皮肤滤泡淋巴瘤（PCFL）（= 26）、边缘区淋巴瘤（PCMZL）（= 25）或未定义的 PCBCL（= 1）患者的数据。在几乎一半的患者中，诊断时存在瘙痒或疼痛。病变主要位于头部和躯干。疾病以单发浸润或播散性病变的形式存在，频率相似。手术、放疗、利妥昔单抗单药或联合化疗分别作为一线治疗应用于 33%、25%、21%和 21%的患者，分别有 94%、83%、50%和 70%的患者获得完全缓解（CR）（= 0.28）。中位缓解持续时间（DoR）为 65 个月（95%CI 35-155）。中位随访时间为 46 个月（范围：3-225）后，估计 5 年总生存率（OS）和无进展生存率（PFS）分别为 93%和 54%。临床表现与文献数据基本一致，但我们观察到一些差异，包括上肢受累的倾向性更高（25%），PCFCL 中更频繁的多灶性表现（64%）和 PCMZL 中的单灶性表现（70%）。无论治疗方式如何，大多数惰性 PCBCL 患者在一线治疗后均获得 CR（77%）。我们没有发现临床特征对治疗结果有任何影响。所有治疗方式均取得了较高的总体缓解率。手术和/或放疗是局限性疾病患者的最佳治疗选择。全身性疾病的治疗应限于疾病的全身性形式。高缓解率、长缓解期和良好的长期生存证实了 PCFCL 和 PCMZL 确实具有惰性特征。
