Kaneko Hitomi, Matsumoto Masanori, Okamoto Kohei, Chyonabayashi Kazuhisa, Hishizawa Masakatsu, Watanabe Mitsumasa, Fujimura Yoshihiro, Tsudo Mitsuru
Third Department of Internal Medicine, Osaka Red Cross Hospital.
Rinsho Ketsueki. 2007 Feb;48(2):144-7.
A 26-year-old man was referred to our hospital with vomiting and high fever. He was disoriented and laboratory results showed microangiopathic hemolytic anemia (Hb 7.1 g/dl) and severe thrombocytopenia (15 x 10(3)/microl). The diagnosis of thrombotic thrombocytopenic purpura (TTP) was established. The activity of von Willebrand cleaving protease (ADAMTS13) was found to be remarkably low (<0.5%) and the high activity of the inhibitor (11.0 Bethesda U/ml) was detected, confirming the diagnosis of typical acquired TIP. Although he had been successfully treated with daily plasma exchange and methylprednisolone, he relapsed after a week. To this therapeutic strategy we added four weekly doses of rituximab (375 mg/m2) and two weekly doses of vincristine (1 mg/m2) simultaneously. The response was very rapid and complete, that is, the platelet count recovered to normal seven days after the first rituximab and vincristine treatment. The patient maintains complete remission nine months later under the administration of 17.5 mg prednisolone. Recovery of the plasma ADAMTS13 activity was clearly correlated with the decrease or disappearance of the inhibitor activity. Combination of rituximab and vincristine therapy would appear to be very effective against refractory TTP.
一名26岁男性因呕吐和高热被转诊至我院。他意识不清，实验室检查结果显示微血管病性溶血性贫血（血红蛋白7.1 g/dl）和严重血小板减少（15×10³/微升）。血栓性血小板减少性紫癜（TTP）诊断成立。发现血管性血友病因子裂解蛋白酶（ADAMTS13）活性显著降低（<0.5%），并检测到抑制剂活性较高（11.0贝塞斯达单位/毫升），确诊为典型的获得性TIP。尽管他通过每日血浆置换和甲泼尼龙治疗取得了成功，但一周后复发。我们在该治疗方案中同时增加了四周剂量的利妥昔单抗（375 mg/m²）和两周剂量的长春新碱（1 mg/m²）。反应非常迅速且完全，即首次使用利妥昔单抗和长春新碱治疗七天后血小板计数恢复正常。九个月后，患者在服用17.5毫克泼尼松龙的情况下维持完全缓解。血浆ADAMTS13活性的恢复与抑制剂活性的降低或消失明显相关。利妥昔单抗和长春新碱联合治疗似乎对难治性TTP非常有效。
