Anthracycline-based chemotherapy as primary treatment for intravascular lymphoma
Anthracycline-based chemotherapy as primary treatment for intravascular lymphoma
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DOI：
10.1093/annonc/mdh274
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发表时间：
2004-08-01
影响因子：
50.5
通讯作者：
Ponzoni, M
中科院分区：
文献类型：
--
作者：
Ferreri, AJM;Campo, E;Ponzoni, M
背景：血管内淋巴瘤(IVL)的最佳治疗缺乏准确的指导方针。患者和方法：回顾38例HIV阴性的IVL患者的临床病理特征，以确定化疗对这些恶性肿瘤的疗效。结果：中位年龄70岁(34~90岁)，男女之比为0.9；有23例(61%)患者有东部肿瘤协作组表现状态(ECOG-PS)；21例(55%)有全身症状。皮肤损害和贫血在接受化疗的患者中明显更常见；中枢神经系统(CNS)和肾脏受累在未经治疗的患者中明显更常见。化疗的有效率为59%，3年总存活率为33+/-11%。6例中枢神经系统受累患者中5例接受化疗，其中4例早期死亡；仅1例接受阿霉素、环磷酰胺、长春新碱、甲氨蝶呤、博莱霉素和强的松龙(MACOP-B)治疗，然后进行大剂量化疗和自体干细胞移植(ASCT)，19个月后存活。另1例患者(43岁，第1期)确诊时接受ASCT支持的大剂量化疗，存活71个月，2例移植后早期死亡的患者在环磷酰胺、阿霉素、长春新碱和强的松(CHOP)治疗后复发。PS小于或等于1、疾病局限于皮肤、分期为1、是否使用化疗与预后相关。结论：以蒽环类药物为主的化疗是IVL的标准治疗方案。然而，存活率令人失望，相关的影响诊断延迟和致命的并发症。对于脑受累的患者，需要更密集的联合用药，其中包含具有较高中枢神经系统生物利用度的药物，ASCT支持的大剂量化疗在具有不良特征的年轻患者中应进一步研究其作用。
Background: Optimal therapeutic management of intravascular lymphoma (IVL) lacks precise guidelines.Patients and methods: The clinico-pathological features of 38 HIV-negative patients with IVL were reviewed to define efficacy of chemotherapy in these malignancies. Clinical characteristics of 22 patients treated with chemotherapy and of 16 untreated patients were compared in order to understand better the impact and causes of potential patient selection.Results: Median age was 70 years (range 34-90), with a male/female ratio of 0.9; 23 (61%) patients had Eastern Cooperative Oncology Group performance status (ECOG-PS) >1;21 (55%) had systemic symptoms. Cutaneous lesions and anemia were significantly more common among patients treated with chemotherapy; central nervous system (CNS) and renal involvement were significantly more common among untreated patients. Chemotherapy was associated with a response rate of 59% and a 3-year overall survival of 33 +/- 11%. Five of six patients with CNS involvement received chemotherapy: four of them died early; only one patient, treated with adriamycin, cyclophosphamide, vincristine, methotrexate, bleomycin and prednisolone (MACOP-B) followed by high-dose chemotherapy and autologous stem cell transplantation (ASCT), was alive at 19 months. High-dose chemotherapy supported by ASCT was indicated at diagnosis in another patient (43 years of age, stage 1), who was alive at 71 months, and at relapse after cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) in two patients who died early after transplantation. PS less than or equal to 1, disease limited to the skin, stage 1, and use of chemotherapy were independently associated with better outcome.Conclusions: Anthracycline-based chemotherapy is the standard treatment for IVL. However, survival is disappointing, with a relevant impact of diagnostic delay and lethal complications. More intensive combinations, containing drugs with higher CNS bioavailability, are needed in cases with brain involvement, and the role of high-dose chemotherapy supported by ASCT should be further investigated in younger patients with unfavorable features.