Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Orbit. 2022 Feb;41(1):97-104. doi: 10.1080/01676830.2021.2018715. Epub 2022 Jan 4.
To compare characteristics of initial ocular adnexal (OA) mantle cell lymphoma (MCL) and initial systemic MCL.
Retrospective, comparative case series. Patients treated for MCL at Mayo Clinic from 1/1/1990 to 11/30/2020. MCL was classified as initial OA if first site was OA or initial systemic if first site was elsewhere with progression or recurrence to the OA region.
Features, treatment, and survival.
There were 50 patients with MCL, 23 initial OA and 27 initial systemic. Patients with initial OA MCL had more conjunctival (52% vs. 19%, p = .017) involvement and less frequently received chemotherapy plus autologous stem cell transplant (ASCT) (9% vs. 33%, p = .046) as initial treatment. Complete remission was achieved in 41 (91% vs. 74%, p = .152) patients. Five-year disease-specific survival was similar in initial OA and initial systemic MCL (92% vs. 83%, p = .187). Subanalysis of patients with initial OA MCL revealed 9 (39%) patients developed tumor recurrence, with mean time to recurrence of 28 months. Comparison (no recurrence vs. recurrence) of initial OA MCL patients revealed those with no recurrence had shorter mean final follow-up (3.3 vs. 9.8 years, p = .005) and more frequent initial treatment with rituximab-based chemotherapy plus ASCT (43% vs. 0%, p = .048). Recurrence had no effect on the 5-year age-adjusted risk of death from lymphoma (HR 2.17, 95% CI 0.55-9.09, p = .266).
Initial OA and initial systemic MCL patients differ in presentation and management but have similar survival.
比较眼部附属器（OA）初发套细胞淋巴瘤（MCL）和全身初发 MCL 的特征。
回顾性、对比病例系列。1990 年 1 月 1 日至 2020 年 11 月 30 日期间在 Mayo 诊所接受 MCL 治疗的患者。如果首个部位为 OA，则将 MCL 归类为初发 OA，如果首个部位为其他部位且随后 OA 区域进展或复发，则将 MCL 归类为初发全身。
特征、治疗和生存。
共有 50 例 MCL 患者，23 例初发 OA，27 例初发全身。初发 OA MCL 患者更常出现结膜受累（52% vs. 19%，p =.017），初治时更常接受化疗联合自体干细胞移植（ASCT）（9% vs. 33%，p =.046）。41 例（91% vs. 74%，p =.152）患者达到完全缓解。初发 OA 和初发全身 MCL 的 5 年疾病特异性生存率相似（92% vs. 83%，p =.187）。初发 OA MCL 患者亚组分析显示，9 例（39%）患者发生肿瘤复发，平均复发时间为 28 个月。无复发的初发 OA MCL 患者与复发患者相比，无复发的患者平均随访时间更短（3.3 年 vs. 9.8 年，p =.005），且更常接受利妥昔单抗为基础的化疗联合 ASCT 治疗（43% vs. 0%，p =.048）。复发对淋巴瘤 5 年年龄调整死亡风险无影响（HR 2.17，95%CI 0.55-9.09，p =.266）。
初发 OA 和初发全身 MCL 患者在表现和治疗上存在差异，但生存率相似。
