Chronic relapsing thrombotic thrombocytopenic purpura in infants with large von Willebrand factor multimers during remission.
Chronic relapsing thrombotic thrombocytopenic purpura in infants with large von Willebrand factor multimers during remission.
复制标题
缓解期间患有大冯维勒布兰德因子多聚体的婴儿的慢性复发性血栓性血小板减少性紫癜。
DOI：
10.1016/s0022-3476(05)80596-7
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发表时间：
1992
期刊：
影响因子：
--
通讯作者：
Steuber,CP
中科院分区：
文献类型：
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作者：
Chintagumpala,MM;Hurwitz,RL;Moake,JL;Mahoney,DH;Steuber,CP
我们研究了两名从新生儿期开始患有复发性片细胞性溶血性贫血和血小板减少症的儿童。一名患者在血栓性血小板减少性紫癜的一次发作期间发生中风。两名儿童在临床和血液学缓解期间均发现存在异常大的血管性血友病因子多聚体。一名接受皮质类固醇治疗和静脉注射免疫球蛋白的患者没有成功。在输注新鲜冰冻血浆后，两名患者的病情立即得到改善。如果没有定期输注新鲜冰冻血浆，血小板减少症的症状会定期复发。其中一名儿童显然患有慢性复发性血栓性血小板减少性紫癜；第二种患有类似于血栓性血小板减少性紫癜的慢性复发性疾病。
We studied two children with recurrent schistocytic hemolytic anemia and thrombocytopenia beginning in the neonatal period. One patient had a stroke during one of the episodes of thrombotic thrombocytopenic purpura. The presence of unusually large von Willebrand factor multimers was demonstrated in both children during clinical and hematologic remissions. Treatment with corticosteroids and intravenous injections of immune globulin was unsuccessful in the one patient who received it. Immediate improvement occurred in both patients after the infusion of fresh-frozen plasma. Symptoms of thrombocytopenia continue to recur at regular intervals in the absence of periodic fresh-frozen plasma infusions. One of these children apparently has chronic relapsing thrombotic thrombocytopenic purpura; the second has a chronic relapsing disorder similar to thrombotic thrombocytopenic purpura.