Rose M, Eldor A
Department of Hematology, Hadassah University Hospital, Jerusalem, Israel.
Am J Med. 1987 Sep;83(3):437-44. doi: 10.1016/0002-9343(87)90753-4.
Described in this study are 38 patients who received treatment for thrombotic thrombocytopenic purpura in 15 hospitals in Israel and the New York City area since 1977, when plasma therapy was introduced. Thirty-seven patients received plasma therapy and 30 survived. In 12 patients (37 percent of survivors), relapsing thrombotic thrombocytopenic purpura developed, manifested by thrombocytopenia and microangiopathic hemolytic anemia, and less frequently by neurological or renal abnormalities. Six patients had a second relapse, and two had five relapses. To assess the severity of the disease, a scoring system was designed based on the four major manifestations of thrombotic thrombocytopenic purpura listed above. The patients who died had a significantly higher score than those who survived. The initial episodes and the relapses of patients with relapsing thrombotic thrombocytopenic purpura were milder than those in patients who only had a single episode and survived. Two relapses, however, were fatal, demonstrating that relapsing thrombotic thrombocytopenic purpura is not a benign disorder. Infections, pregnancy and surgery were frequently associated with the initial episodes and the relapses. Hence, patients who recover from thrombotic thrombocytopenic purpura should be alerted to the possibility of relapse in association with these conditions.
本研究描述了自1977年引入血浆疗法以来，在以色列和纽约市地区的15家医院接受血栓性血小板减少性紫癜治疗的38例患者。37例患者接受了血浆疗法，30例存活。在12例患者（占存活者的37%）中，出现了复发性血栓性血小板减少性紫癜，表现为血小板减少和微血管病性溶血性贫血，较少表现为神经或肾脏异常。6例患者出现第二次复发，2例患者出现5次复发。为评估疾病的严重程度，基于上述血栓性血小板减少性紫癜的四种主要表现设计了一个评分系统。死亡患者的评分显著高于存活患者。复发性血栓性血小板减少性紫癜患者的初始发作和复发比仅发作一次且存活的患者更为轻微。然而，有两次复发是致命的，这表明复发性血栓性血小板减少性紫癜并非良性疾病。感染、妊娠和手术常与初始发作及复发相关。因此，从血栓性血小板减少性紫癜中康复的患者应警惕与这些情况相关的复发可能性。
