Fløisand Yngvar, Brinch Lorentz, Gedde-Dahl Tobias, Tjønnfjord Geir E
Seksjon for blodsykdommer, Medisinsk avdeling, Rikshospitalet, 0027 Oslo.
Tidsskr Nor Laegeforen. 2004 Mar 18;124(6):768-70.
T-cell prolymphocytic leukaemia (T-PLL) is a rare post-thymic T-cell malignancy with an aggressive clinical course. It has generally been resistant to alkylating chemotherapy, but some effect has been observed with the purine analog 2-deoxycoformicin with documented partial or complete response rates in up to 45% of patients. Treatment with monoclonal antibodies against CD 52 has been shown to be highly effective in T-PLL with response rates of up to 76%. This may allow for further consolidating treatment with high-dose chemotherapy with autologous stem cell support or allogeneic stem cell transplantation.
Five patients treated at Rikshospitalet University Hospital are evaluated and the literature on T-PLL is reviewed.
Four of our patients were treated with alemtuzumab. Three showed complete or partial response. One patient underwent allogeneic stem cell transplantation with an HLA-identical sibling, but died on day 21 as a result of transplantation complications. The treatment is generally well tolerated; the principal management problem is immunosuppression, as shown in one patient who developed a varicella-zoster meningoencephalomyelitis as a consequence of not receiving antiviral prophylaxis. The main infusion-related adverse effects are fever and chills.
T 细胞原淋巴细胞白血病(T-PLL)是一种罕见的胸腺后 T 细胞恶性肿瘤,临床病程侵袭性强。它通常对烷化剂化疗耐药,但嘌呤类似物 2-脱氧助间型霉素已观察到有一定疗效,有记录显示高达 45%的患者有部分或完全缓解率。已证明用抗 CD52 单克隆抗体治疗 T-PLL 非常有效,缓解率高达 76%。这可能允许采用高剂量化疗并辅以自体干细胞支持或异基因干细胞移植进行进一步巩固治疗。
对在里克斯医院大学医院接受治疗的 5 例患者进行评估,并复习有关 T-PLL 的文献。
我们的 4 例患者接受了阿仑单抗治疗。3 例显示完全或部分缓解。1 例患者与 HLA 相同的同胞进行了异基因干细胞移植,但因移植并发症于第 21 天死亡。该治疗一般耐受性良好;主要的管理问题是免疫抑制,如 1 例患者因未接受抗病毒预防而发生水痘-带状疱疹脑膜脑脊髓炎所示。主要的输液相关不良反应是发热和寒战。