妊娠期重度特发性血小板减少性紫癜

妊娠期特发性血小板减少性紫癜（idiopathic thrombocytopenic purpura,ITP）是一种自身免疫性疾病,是妊娠早期血小板减少的最常见原因,由于妊娠妇女血浆中的抗血小板抗体与血小板特异性结合,以及T细胞基因表达缺陷,导致巨核细胞成熟障碍、血小板破坏增加及生成不足,其确切发病机制尚未明确。妊娠期重度ITP增加了母儿不良妊娠结局的发生风险,如妊娠期自发性出血、妊娠期贫血、产后出血、早产及新生儿血小板减少症等。目前临床上妊娠期重度ITP的处理较为棘手,一线治疗方案主要是口服糖皮质激素和静脉注射免疫球蛋白,然而在一线治疗无效的情况下,有效的二线治疗和及时的产科处理变得尤为重要,如脾切除术、输注血小板及血小板生成素类药物等。本文结合近年文献对妊娠期重度ITP的发病机制、妊娠结局及治疗等方面进行综述。

Severe Idiopathic Thrombocytopenic Purpura in Pregnant Women

Idiopathic thrombocytopenic purpura(ITP)in pregnancy is an autoimmune disease,which is the most common cause of thrombocytopenia in early pregnancy.Due to the specific binding of antiplatelet antibodies in maternal plasma to platelets and the deficiency of T cells gene expression,the megakaryocyte maturation disorder,increased platelet destruction and insufficient production are induced.However,the exact pathogenesis is not clear yet.Severe ITP increases the risk of adverse pregnancy outcomes of mothers and infants,such as spontaneous haemorrhage during pregnancy,anemia,postpartum hemorrhage,premature birth and neonatal thrombocytopenia.At present,it is difficult to deal with severe ITP.The first-line treatment is mainly oral corticosteroids and intravenous immunoglobulin.When the first-line treatment fails,the effective second-line treatment and timely obstetric treatment will be particularly important,for example,splenectomy,platelet transfusion and thrombopoietin drugs,etc.This review summarized the pathogenesis,treatment and the maternal and infant outcomes of severe ITP during pregnancy.