Department of Pathology, The Warren Alpert Medical School at Brown University, Rhode Island Hospital, Providence, RI 02903, USA.
Clin Lymphoma Myeloma Leuk. 2010 Dec;10(6):480-3. doi: 10.3816/CLML.2010.n.084.
Acute T-cell leukemia/lymphoma (ATLL) is a post thymic (peripheral) T-cell neoplasm caused by human T-cell lymphotropic virus type 1 (HTLV-1). Historically, the chemotherapy regimen CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) has been the standard treatment of this rare malignancy. However, its prognosis is poor and median survival in the aggressive variants of ATLL is only 6-10 months. Recently, a more aggressive regimen piloted in Japan, vincristine/cyclophosphamide/doxorubicin/prednisone (VCAP)- doxorubicin/ranimustine/prednisone (AMP)- vindesine/etoposide/carboplatin/prednisone (VECP), has been reported to yield better survival results over biweekly CHOP in a phase III trial. However, the hyper- cyclophosphamide/vincristine/doxorubicin/dexamethasone (CVAD) regimen is a much more frequently used regimen for the treatment of aggressive hematologic malignancies, and has a higher intensity then CHOP. Yet, there is little reported experience with hyper-CVAD regimen in ATLL.
We present 2 patients diagnosed with ATLL who were treated with hyper-CVAD chemotherapy and have achieved a durable complete remission. One of the patients has gone on to receive an allogeneic bone marrow transplantation and has been in complete remission for over 18 months. The other has been in a continuous remission for approximately 12 months. We also review the past published experience with the hyper-CVAD regimen in patients with ATLL.
A commonly used chemotherapy regimen for aggressive hematologic malignancies, hyper-CVAD, can induce durable remissions in patients with ATLL.
急性 T 细胞白血病/淋巴瘤（ATLL）是一种由人类 T 细胞白血病病毒 1 型（HTLV-1）引起的胸腺后（外周）T 细胞肿瘤。历史上，CHOP（环磷酰胺/多柔比星/长春新碱/泼尼松）化疗方案一直是这种罕见恶性肿瘤的标准治疗方法。然而，其预后较差，侵袭性 ATLL 变异型的中位生存期仅为 6-10 个月。最近，在日本试行的一种更为激进的方案，长春新碱/环磷酰胺/多柔比星/泼尼松（VCAP）-多柔比星/雷那米特/泼尼松（AMP）-长春碱/依托泊苷/卡铂/泼尼松（VECP），在一项 III 期试验中报告称，与每周两次的 CHOP 相比，生存结果更好。然而，高剂量环磷酰胺/长春新碱/多柔比星/地塞米松（CVAD）方案是治疗侵袭性血液系统恶性肿瘤更常用的方案，其强度高于 CHOP。然而，关于 CVAD 方案在 ATLL 中的应用经验却很少。
我们报告了 2 例诊断为 ATLL 的患者，他们接受了高剂量 CVAD 化疗，并获得了持久的完全缓解。其中 1 例患者接受了异基因骨髓移植，已完全缓解超过 18 个月。另 1 例患者持续缓解约 12 个月。我们还回顾了过去发表的关于 ATLL 患者使用 CVAD 方案的经验。
高剂量 CVAD 是一种常用于治疗侵袭性血液系统恶性肿瘤的化疗方案，可诱导 ATLL 患者持久缓解。
