Mocikova Heidi, Pytlík Robert, Benesova Katerina, Janikova Andrea, Duras Juraj, Sykorova Alice, Steinerova Katerina, Prochazka Vit, Campr Vit, Belada David, Trneny Marek
Department of Internal Medicine - Hematology, Third Faculty of Medicine, Charles University, Prague, Czechia.
Institute of Haematology and Blood Transfusion, Prague, Czechia.
Front Oncol. 2022 May 12;12:874462. doi: 10.3389/fonc.2022.874462. eCollection 2022.
We analyzed the incidence, risk factors of central nervous system (CNS) relapse, and outcome of CNS involvement in patients with peripheral T-cell lymphomas (PTCL) from the Czech Lymphoma Study Group Registry NiHiL (Clinical Trial gov. NCT03199066).
Out of 1,040 patients with PTCL, we identified 29 patients (2.79%) with CNS involvement: 2 patients with primary CNS T cell lymphoma, 11 patients with CNS and systemic disease at diagnosis, and 16 patients (1.54%) at CNS relapse. The most common histology with CNS disease was PTCL, not otherwise specified. Progression-free survival (PFS) was defined as the time interval from diagnosis to progression or death. PFS-2 was defined as the interval from the date of a new relapse until the next relapse.
Patients with testicular involvement received intrathecal prophylaxis with methotrexate. High-dose methotrexate-based treatment was administered in 44.8% of patients with CNS disease. Median follow-up was 71.3 months. The difference between the median PFS of 1,027 patients without initial CNS disease (32.6 months) and 11 patients with initial CNS and systemic disease (4.8 months) was significant ( = 0.04). The difference between the median PFS2 in CNS relapses (10.1 months) and 493 relapses outside of CNS (9.1 months) was not significant ( = 0.6). Risk factors for CNS relapses included the following: involvement of more than one extranodal site ( = 0.008), soft tissue involvement ( = 0.003), testicular involvement ( = 0.046), and the presence of B symptoms ( = 0.035). The difference between the median OS of 1,027 patients without initial CNS disease (46.0 months) and 11 patients with initial CNS and systemic disease (18.2 months) was significant ( = 0.02). The median OS2 in CNS relapses was 11.8 months and that in relapses outside of CNS was 21.3 months. CNS involvement was not associated with a significantly worse OS compared to relapsed/refractory patients without CNS involvement ( = 0.1).
The incidence of CNS disease at the time of diagnosis and at relapse in PTCL is low and usually associated with other systemic involvement. The prognosis of PTCL with initial CNS involvement is significantly worse when compared to patients without CNS disease at diagnosis. The outcome of CNS relapse is comparable with relapsed PTCL outside of CNS. The optimal treatment is not defined yet.
我们分析了捷克淋巴瘤研究组注册中心NiHiL（临床试验.gov NCT03199066）中，外周T细胞淋巴瘤（PTCL）患者中枢神经系统（CNS）复发的发生率、危险因素及CNS受累的结局。
在1040例PTCL患者中，我们确定了29例（2.79%）有CNS受累：2例原发性CNS T细胞淋巴瘤，11例诊断时CNS和全身疾病患者，以及16例（1.54%）CNS复发患者。CNS疾病最常见的组织学类型为未另行特指的PTCL。无进展生存期（PFS）定义为从诊断到疾病进展或死亡的时间间隔。PFS-2定义为从新复发日期到下一次复发的时间间隔。
睾丸受累患者接受了鞘内注射甲氨蝶呤预防。44.8%的CNS疾病患者接受了基于大剂量甲氨蝶呤的治疗。中位随访时间为71.3个月。1027例无初始CNS疾病患者的中位PFS（中位数为32.6个月）与11例初始CNS和全身疾病患者的中位PFS（4.8个月）之间的差异具有统计学意义（P = 0.04）。CNS复发患者的中位PFS2（10.1个月）与493例CNS外复发患者的中位PFS2（9.1个月）之间的差异无统计学意义（P = 0.6）。CNS复发的危险因素包括：超过一个结外部位受累（P = 0.008）；软组织受累（P = 0.003）；睾丸受累（P = 0.046）；以及存在B症状（P = 0.035）。1027例无初始CNS疾病患者的中位总生存期（OS）（46.0个月）与11例初始CNS和全身疾病患者的中位OS（18.2个月）之间的差异具有统计学意义（P = 0.02）。CNS复发患者的中位OS2为11.8个月，CNS外复发患者的中位OS2为21.3个月。与无CNS受累的复发/难治性患者相比，CNS受累患者的OS并无显著更差（P = 0.1）。
PTCL诊断时和复发时CNS疾病的发生率较低，且通常与其他全身受累相关。与诊断时无CNS疾病的患者相比，初始CNS受累的PTCL预后明显更差。CNS复发的结局与CNS外复发的PTCL相当。最佳治疗方案尚未确定。
