Rodella Elisabetta, Pacquola Enrica, Bianchini Enzo, Ramazzina Emilio, Paolini Rossella
Department of Oncology and Oncohaematology, S Maria della Misericordia Hospital, Rovigo, Italy.
Blood Coagul Fibrinolysis. 2008 Jun;19(4):315-8. doi: 10.1097/MBC.0b013e3282f9ae12.
We describe a 58-year-old woman affected by immune thrombocytopenic purpura (ITP) since 1999, well controlled by low doses of steroid for 4 years, who experienced a relapse with severe mixed type Evans syndrome in March 2006. After an initial response to high doses of steroid, severe anaemia recurred 2 months later, this time resistant to second-line therapy with intravenous immunoglobulins (IVIG) and cyclophosphamide. So in May, we started the treatment with anti-CD20 monoclonal antibody rituximab with the dose of 375 mg/m2 once weekly for a total of four doses. We obtained a full normalization of haemoglobin concentration, but the disease haemolytic parameters persisted. Therefore, we decided to treat the patient with two monthly courses of rituximab, and a gradual normalization of haptoglobin and lactate dehydrogenase (LDH) plasma levels was finally achieved, with a sustained response up to date, lasting more than 12 months. We conclude that rituximab treatment is effective in refractory patients with mixed type Evans syndrome, and consolidation therapy should be considered to prolong beneficial effects achieved during the induction.
我们描述了一位58岁的女性，自1999年起患有免疫性血小板减少性紫癜（ITP），4年来通过低剂量类固醇得到良好控制，2006年3月病情复发，出现严重的混合型伊文氏综合征。在对高剂量类固醇产生初始反应后，2个月后严重贫血复发，此次对静脉注射免疫球蛋白（IVIG）和环磷酰胺的二线治疗耐药。于是在5月，我们开始用抗CD20单克隆抗体利妥昔单抗治疗，剂量为375 mg/m²，每周一次，共四剂。我们使血红蛋白浓度完全恢复正常，但疾病的溶血参数仍然存在。因此，我们决定用两个疗程的利妥昔单抗每月治疗该患者一次，最终实现了触珠蛋白和乳酸脱氢酶（LDH）血浆水平的逐渐正常化，至今仍有持续反应，持续超过12个月。我们得出结论，利妥昔单抗治疗对难治性混合型伊文氏综合征患者有效，应考虑巩固治疗以延长诱导治疗期间取得的有益效果。
