Advani Ranjana, Horwitz Steven, Zelenetz Andrew, Horning Sandra J
Stanford University Medical Center, Stanford, CA 94305-5821, USA.
Leuk Lymphoma. 2007 Mar;48(3):521-5. doi: 10.1080/10428190601137658.
Angioimmunoblastic T cell lymphoma is a distinct entity for which there is no standard therapy. On the basis of the rationale that CsA may represent a novel drug for AITL, a disease with considerable immune dysregulation, and encouraging case reports, the authors have treated 12 patients with this agent. Ten had failed prior steroids and/or chemotherapy and two had no prior therapy. CsA was administered at a dose of 3 - 5 mg/kg PO bid for 6 - 8 weeks and gradually tapered by 50 mg every 1 - 3 weeks. Responding patients received a maintenance dose of 50 - 100 mg, with a gradual taper after a maximal response was achieved as tolerated. Doses were titrated for renal dysfunction or hypertension. CsA levels were not monitored. Eight of 12 patients responded (three complete and five partial remissions). Dose reductions were required in six patients; renal insufficiency (n = 3), fatigue (n = 2), and hypertension (n = 1). Two patients developed acute infections and one patient died shortly after active treatment. These results suggest that CsA deserves further testing as a novel therapy for AITL. By interrupting T-cell activation, CsA may alter the immune dysregulation that characterizes AILT. The efficacy of CsA is being explored in patients with recurrent AILT in a prospective trial (ECOG 2402).
血管免疫母细胞性T细胞淋巴瘤是一种尚无标准治疗方法的独特疾病。基于环孢素A（CsA）可能是治疗血管免疫母细胞性T细胞淋巴瘤（AITL）的一种新药的理论基础，该疾病存在相当程度的免疫失调，且有令人鼓舞的病例报告，作者用该药治疗了12例患者。其中10例之前使用类固醇和/或化疗失败，2例未曾接受过治疗。CsA的给药剂量为3 - 5mg/kg口服，每日2次，持续6 - 8周，然后每1 - 3周逐渐减量50mg。有反应的患者接受50 - 100mg的维持剂量，在达到最大反应后根据耐受情况逐渐减量。根据肾功能不全或高血压情况调整剂量。未监测CsA水平。12例患者中有8例有反应（3例完全缓解，5例部分缓解）。6例患者需要减量；原因包括肾功能不全（3例）、疲劳（2例）和高血压（1例）。2例患者发生急性感染，1例患者在积极治疗后不久死亡。这些结果表明，CsA作为AITL的一种新疗法值得进一步试验。通过阻断T细胞活化，CsA可能改变AITL所特有的免疫失调。目前正在一项前瞻性试验（ECOG 2402）中探索CsA对复发性AITL患者的疗效。
