Jahnke Kristoph, Korfel Agnieszka, Komm Julia, Bechrakis Nikolaos E, Stein Harald, Thiel Eckhard, Coupland Sarah E
Department of Haematology, Oncology and Transfusion Medicine, Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, 12200 Berlin, Germany.
Graefes Arch Clin Exp Ophthalmol. 2006 Jun;244(6):663-9. doi: 10.1007/s00417-005-0138-9. Epub 2005 Oct 16.
The prognosis of intraocular lymphoma (IOL) is poor, and the optimal treatment has yet to be defined. This study assesses the clinical characteristics and outcome of patients with IOL diagnosed and treated in the new millennium.
Patient data in this retrospective multicentre study were compiled by standardised questionnaires sent to seven university ophthalmology departments. All cases diagnosed with primary and secondary IOL in the past 5 years not associated with HIV infection were included.
Twenty-two patients, 11 men and women; median age 64 (range 38-83) years, median Karnofsky performance status 90% (range 50-100%), were included. Nineteen patients had primary IOL (PIOL): 13 a newly diagnosed disease and six an ocular relapse of primary central nervous system lymphoma (PCNSL). Three patients had secondary IOL. First-line treatment for IOL included systemic chemotherapy in 13 cases, ocular radiation in six and intraocular chemotherapy in three. Complete remission was achieved in 14/20 evaluable patients, partial remission in five and stable disease in one. All patients treated with ifosfamide (IFO) or trofosfamide (TRO) (n=8) responded. Median progression-free survival (PFS) and overall survival were 10 (range 1+ to 44.5+) and 22.5 (range 1+ to 49+) months, respectively. Patients with newly diagnosed PIOL and ocular relapse of PCNSL had a median PFS of 10 (range 1+ to 44.5+) and 6 (range 2 to 6+) months, respectively. Median PFS was 12 (range 3+ to 22.5+) months after systemic and 5.5 (range 1+ to 44.5+) months after local first-line therapy.
The prognosis of PIOL is similar to that of PCNSL without ocular involvement. Systemic therapy possibly prolongs PFS as compared with local management of (P)IOL. The high response rate to monotherapy with IFO and TRO is promising.
眼内淋巴瘤（IOL）的预后较差，最佳治疗方案尚未确定。本研究评估了新千年诊断和治疗的IOL患者的临床特征和预后。
本回顾性多中心研究的患者数据通过发送给七个大学眼科部门的标准化问卷收集。纳入所有在过去5年中诊断为原发性和继发性IOL且与HIV感染无关的病例。
共纳入22例患者，男女各11例；中位年龄64岁（范围38 - 83岁），中位卡诺夫斯基功能状态为90%（范围50 - 100%）。19例患者为原发性IOL（PIOL）：13例为新诊断疾病，6例为原发性中枢神经系统淋巴瘤（PCNSL）的眼部复发。3例患者为继发性IOL。IOL的一线治疗包括13例全身化疗、6例眼部放疗和3例眼内化疗。20例可评估患者中，14例达到完全缓解，5例部分缓解，1例病情稳定。所有接受异环磷酰胺（IFO）或曲磷胺（TRO）治疗的患者（n = 8）均有反应。中位无进展生存期（PFS）和总生存期分别为10个月（范围1 +至44.5 +）和22.5个月（范围1 +至49 +）。新诊断PIOL和PCNSL眼部复发患者的中位PFS分别为10个月（范围1 +至44.5 +）和6个月（范围2至6 +）。全身一线治疗后的中位PFS为12个月（范围3 +至22.5 +），局部一线治疗后的中位PFS为5.5个月（范围1 +至44.5 +）。
PIOL的预后与无眼部受累的PCNSL相似。与（P）IOL的局部治疗相比，全身治疗可能延长PFS。IFO和TRO单药治疗的高反应率很有前景。
