Savignano Chiara, Rinaldi Cristina, De Angelis Vincenzo
Therapeutic Apheresis Unit and Stem Cell Manipulation Laboratory, Department of Transfusion Medicine, Azienda Ospedaliero Universitaria, Udine, Italy.
Therapeutic Apheresis Unit and Stem Cell Manipulation Laboratory, Department of Transfusion Medicine, Azienda Ospedaliero Universitaria, Udine, Italy.
Transfus Apher Sci. 2015 Dec;53(3):262-8. doi: 10.1016/j.transci.2015.11.005. Epub 2015 Nov 25.
Thrombotic thrombocytopenic purpura (TTP) is a severe, life-threatening disease that needs urgent diagnosis and prompt therapeutic intervention. In pregnant women TTP may complicate the course of gestation putting mother and child at vital risk. Differential diagnosis with other obstetric and medical disorders may be difficult due to the overlap of several clinical and laboratory findings. Our understanding of the pathophysiology of TTP has allowed ADAMTS13 testing to have a central role in confirming the clinical diagnosis but the main limitation is that an ADAMTS13 assay is not available in "real time". Here we report the clinical course and treatment outcome of two young women with clinical manifestations of pregnancy associated TTP and briefly discuss the main topics of disease diagnosis and management.
血栓性血小板减少性紫癜(TTP)是一种严重的、危及生命的疾病,需要紧急诊断和及时的治疗干预。在孕妇中,TTP可能使妊娠过程复杂化,使母婴面临重大风险。由于一些临床和实验室检查结果存在重叠,与其他产科和内科疾病进行鉴别诊断可能会很困难。我们对TTP病理生理学的认识使ADAMTS13检测在确诊临床诊断中发挥核心作用,但主要局限性在于无法“实时”进行ADAMTS13检测。在此,我们报告两名有妊娠相关TTP临床表现的年轻女性的临床病程及治疗结果,并简要讨论疾病诊断和管理的主要问题。