Eskelund Christian Winther, Dimopoulos Kostas, Kolstad Arne, Glimelius Ingrid, Räty Riikka, Gjerdrum Lise Mette Rahbek, Sonnevi Kristina, Josefsson Pär, Nilsson-Ehle Herman, Bentzen Hans H N, Fagerli Unn Merete, Kuittinen Outi, Haaber Jacob, Niemann Carsten Utoft, Pedersen Lone Bredo, Larsen Maria Torp, Geisler Christian Hartmann, Hutchings Martin, Jerkeman Mats, Grønbæk Kirsten
Department of Haematology, Rigshospitalet, Copenhagen, Denmark.
Biotech Research & Innovation Centre BRIC, University of Copenhagen, Denmark.
Hemasphere. 2020 Dec 21;5(1):e510. doi: 10.1097/HS9.0000000000000510. eCollection 2021 Jan.
Mantle cell lymphoma (MCL) is an incurable disease with a highly variable clinical course. The prognosis after relapse is generally poor, and no standard of care exists. We investigated the postrelapse outcomes of 149 patients who were initially treated in the Nordic Lymphoma Group trials, MCL2 or MCL3, both representing intensive cytarabine-containing frontline regimens including autologous stem cell transplant. Patients with progression of disease before 24 months (POD24, n = 51, 34%) displayed a median overall survival of 6.6 months compared with 46 months for patients with later POD (n = 98, 66%; < 0.001). MCL international prognostic index, cell proliferation marker, blastoid morphology, and mutations showed independent prognostic value irrespective of POD24, and in a combined, exploratory risk score, patients with 0, 1, 2-3, or 4-5 high-risk markers, respectively, displayed a 5-year overall survival of 62%, 39%, 31%, and 0%. By a comparison of median progression-free survival of the different salvage therapies in the relapse setting, bendamustine-rituximab was superior to all other combination chemotherapy regimens; however, it was also associated with longer responses to last line of therapy. Collectively, we confirm the prognostic impact of POD24 and highlight the relevance of other biomarkers, and we emphasize the importance of novel therapies for patients with high-risk features at first POD.
套细胞淋巴瘤（MCL）是一种无法治愈的疾病，临床病程高度可变。复发后的预后通常较差，且不存在标准的治疗方案。我们调查了149例最初在北欧淋巴瘤组试验MCL2或MCL3中接受治疗的患者复发后的结局，这两项试验均代表含阿糖胞苷的强化一线方案，包括自体干细胞移植。24个月前疾病进展的患者（POD24，n = 51，34%）的中位总生存期为6.6个月，而POD较晚的患者（n = 98，66%；P<0.001）为46个月。MCL国际预后指数、细胞增殖标志物、母细胞样形态和突变显示出独立的预后价值，与POD24无关，并且在一个综合的探索性风险评分中，分别具有0、1、2 - 3或4 - 5个高危标志物的患者的5年总生存率分别为62%、39%、31%和0%。通过比较复发情况下不同挽救疗法的中位无进展生存期，苯达莫司汀 - 利妥昔单抗优于所有其他联合化疗方案；然而，它也与对最后一线治疗的较长反应相关。总体而言，我们证实了POD24的预后影响并强调了其他生物标志物的相关性，并且我们强调了针对首次POD时具有高危特征患者的新型疗法的重要性。
