Nikolaou Marinos, Karakantza Marina, Adonakis George, Theodorou George, Zoumbos Nikolaos, Decavalas George
Department of Obstetrics and Gynecology, University Hospital, Medical School of Patras, Greece.
Med Pregl. 2012 Sep-Oct;65(9-10):436-9. doi: 10.2298/mpns1210436n.
Thrombotic thrombocytopenic purpura is a rare life-threatening disorder characterized by thrombocytopenia and microangiopathic hemolytic anemia. It is caused by the absent or severe deficiency of the von Willebrand Factor-cleaving protease named ADAMTS13. Pregnancy is a well recognized factor precipitating the appearance of the disease both in women that had reduced levels of ADAMTS13 activity prior to gestation and in those with other inherited or acquired thrombophilic syndromes.
We report a 25-year old woman with severe ADAMTS13 deficiency presented early in her 1st pregnancy and relapsed in two subsequent gestations. This presentation is uncommon for thrombotic thrombocytopenic purpura is associated with pregnancy (ADAMTS13 deficiency < 5%, without an inhibitor). In the first pregnancy she started with daily plasma exchange 1.5 x volume, corticosteroids and IV immunoglobulin and finally entered remission after 23 sessions and termination of pregnancy. In the second pregnancy she did not receive prophylactic treatment and relapsed in the 3rd trimester. Prophylactic treatment during the third pregnancy with plasma infusions proved also ineffective to prevent relapse.
Many issues regarding treatment and prevention of thrombotic thrombocytopenic purpura relapses in subsequent pregnancies are unclear. Proposed guidelines recommend that the same treatment should be performed on pregnant and non pregnant patients without modification of plasma replacement dose according to ADAMTS13 levels. In addition, many authors suggest that pregnant patients with history of thrombotic thrombocytopenic purpura and severe deficiency of ADAMTS13 levels should received prophylactic treatment for prevention of relapses in the subsequent pregnancies.
Severe ADAMTS 13 deficiency may present as thrombotic thrombocytopenic purpura of pregnancy. Pregnant women with thrombotic thrombocytopenic purpura and especially with severe deficiency of ADAMTS13 levels require specific consideration regarding treatment and prophylaxis in subsequent pregnancies.
血栓性血小板减少性紫癜是一种罕见的危及生命的疾病，其特征为血小板减少和微血管病性溶血性贫血。它是由名为ADAMTS13的血管性血友病因子裂解蛋白酶缺乏或严重不足引起的。妊娠是一个公认的促使该疾病出现的因素，无论是在妊娠前ADAMTS13活性水平降低的女性中，还是在患有其他遗传性或获得性血栓形成倾向综合征的女性中。
我们报告一名25岁严重ADAMTS13缺乏的女性，在其首次妊娠早期发病，并在随后的两次妊娠中复发。这种表现对于与妊娠相关的血栓性血小板减少性紫癜（ADAMTS13缺乏<5%，无抑制剂）来说并不常见。在首次妊娠中，她开始每日进行1.5倍血容量的血浆置换、使用皮质类固醇和静脉注射免疫球蛋白，最终在进行23次治疗并终止妊娠后进入缓解期。在第二次妊娠中，她未接受预防性治疗，并在妊娠晚期复发。在第三次妊娠期间进行血浆输注的预防性治疗也未能有效预防复发。
关于血栓性血小板减少性紫癜在后续妊娠中复发的治疗和预防的许多问题尚不清楚。提议的指南建议，对妊娠和非妊娠患者应进行相同的治疗，且不根据ADAMTS13水平调整血浆置换剂量。此外，许多作者建议，有血栓性血小板减少性紫癜病史且ADAMTS13水平严重缺乏的妊娠患者，应接受预防性治疗以预防后续妊娠中的复发。
严重ADAMTS 13缺乏可能表现为妊娠性血栓性血小板减少性紫癜。患有血栓性血小板减少性紫癜尤其是ADAMTS13水平严重缺乏的孕妇，在后续妊娠的治疗和预防方面需要特殊考虑。
