Ozdogu Hakan, Boga Can, Kizilkilic Ebru, Yeral Mahmut, Kozanoglu Ilknur, Karatas Mehmet
Department of Hematology, Baskent University Faculty of Medicine, 06490 Ankara, Turkey.
J Thromb Thrombolysis. 2007 Apr;23(2):147-50. doi: 10.1007/s11239-006-9051-2. Epub 2007 Jan 13.
Refractory condition can occur in 10-30% of all cases of thrombotic thrombocytopenic purpura despite increased frequency of total plasma exchange. Rituximab can affect the clinical outcome of the refractory cases. However, little is known about usefulness of rituximab on central nervous system involvement mimicking acute ischemic stroke.
We report the case of a woman with refractory thrombotic thrombocytopenic purpura who developed an acute onset right sided paralysis, dysarthria, and central facial paralysis, suggestive of cerebrovascular accident while under plasma exchange, corticosteroid, and vincristine therapy.
After initiation of rituximab (375 mg/m(2) weekly for 4 weeks), a dramatic response occurred and the patient's neurologic function recovered fully within days. Sustained remission was achieved, and the patient was well 1 year after her admission, while she was on azathioprine treatment.
This report suggests that rituximab can provide a good outcome of the dramatic central nervous system involvement in patients with thrombotic thrombocytopenic purpura.
尽管全血浆置换频率增加,但在所有血栓性血小板减少性紫癜病例中,10%-30%的患者仍会出现难治性情况。利妥昔单抗可影响难治性病例的临床结局。然而,关于利妥昔单抗对模拟急性缺血性卒中的中枢神经系统受累的有效性知之甚少。
我们报告了一例难治性血栓性血小板减少性紫癜女性患者的病例,该患者在接受血浆置换、皮质类固醇和长春新碱治疗时,急性起病出现右侧偏瘫、构音障碍和中枢性面瘫,提示脑血管意外。
开始使用利妥昔单抗(375mg/m²,每周一次,共4周)后,出现显著反应,患者的神经功能在数天内完全恢复。实现了持续缓解,患者入院1年后情况良好,当时她正在接受硫唑嘌呤治疗。
本报告表明,利妥昔单抗可使血栓性血小板减少性紫癜患者中枢神经系统严重受累的情况获得良好转归。