AN OVERVIEW OF CLINICAL AND PHYSIOPATHOLOGICAL FEATURES OF THROMBOTIC THROMBOCYTOPENIC PURPURA DURING PREGNANCY

Thrombotic thrombocytopenic purpura (TTP), haemolytic-uraemic syndrome (HUS), preeclampsiaHELLP (haemolysis, elevated liver enzymes, low platelet count) syndrome, and some other autoimmune syndromes like catastrophic antiphospholipid syndrome (CAPS), are microangiopathic disorders that can be diagnosed during pregnancy. Although the underlying physiopathological mechanisms differ, the clinical consequences are very similar in all of them, so that it is very difficult to establish a differential diagnosis. Since each disease has its own treatment particularities, and maternal and perinatal morbidity and mortality are high when treatment is not appropriate, gynaecologists need to have a thorough understanding of differentiating characteristics of these disorders. TTP is more common in women, with a peak incidence in the fourth decade of life, and 10% of all cases occur during pregnancy. In the absence of adequate diagnosis and treatment, the maternal and foetal mortality rate approaches 90%. Preconceptional counselling should be provided to women with prior episodes of TTP or congenial Upshaw-Schulman syndrome.

血栓性血小板减少性紫癜（TTP）、溶血尿毒综合征（HUS）、子痫前期 - HELLP（溶血、肝酶升高、血小板计数低）综合征以及其他一些自身免疫性综合征，如灾难性抗磷脂综合征（CAPS），均为微血管病性疾病，可在妊娠期确诊。尽管潜在的病理生理机制不同，但它们的临床后果非常相似，因此很难进行鉴别诊断。由于每种疾病都有其自身的治疗特点，且治疗不当时孕产妇和围产儿的发病率和死亡率都很高，妇科医生需要全面了解这些疾病的鉴别特征。TTP在女性中更为常见，发病高峰在40岁左右，且10%的病例发生在妊娠期。在缺乏充分诊断和治疗的情况下，孕产妇和胎儿的死亡率接近90%。对于既往有TTP发作或先天性Upshaw - Schulman综合征的女性，应提供孕前咨询。