Thrombotic thrombocytopenicpurpura during pregnancy: report of 2 cases and literature review

Abstract

Objective To explore the importance and strategies of early diagnosis and treatment of thrombotic thrombocytopenic purpura (TTP) in pregnancy. Methods Two cases of pregnancy complicated with TTP admitted to our hospital from January 2015 to July 2020 were recruited and retrospectively summarized. The characteristics of the disease, diagnosis and treatment process and prognosis were analyzed to summarize the importance and strategies of early diagnosis and treatment. Related literatures were reviewed. Results The initial symptoms were thrombocytopenia and anemia. Case 1 developed postpartum, and the diagnosis of microangiopathic hemolytic anemia was not timely confirmed by peripheral blood smear, and this resulted in delayed diagnosis, delayed plasma exchange and death of the patient. Case 2 developed in the middle of pregnancy. Broken red blood cells were found on peripheral blood smear in time, and TTP was thus diagnosed. Plasma exchange was performed immediately, and the patient was cured and discharged. There were totally 57 patients in literature review. The results suggested that TTP can occur in all stages of pregnancy, and microangiopathic hemolytic anemia and thrombocytopenia can be the presumptive diagnosis of TTP. TTP triad (microangiopathic hemolytic anemia, thrombocytopenia, and neurological symptoms) were observed in 26 cases (45.6%), of which 5 cases (19.2%) died; 7 cases (12.3%) were combined with pentad (triad+fever and renal dysfunction), including 4 died (57.1%). In the 36 cases tested for the activity of von Willebrand factor lyase, ADAMTS13, 28 cases (77.8%) had severely decreased activity (activity < 10%). The mortality rate in the plasma exchange group (6.8%, 3/44) was significantly lower than that in the group without plasma exchange (69.2%, 9/13, P < 0.05). Conclusion Peripheral blood smear is an important reference in the diagnosis of microangiopathic hemolysis, and the possibility of TTP should be highly vigilant for pregnant women with microangiopathic hemolytic anemia and thrombocytopenia, and plasma exchange as soon as possible after suspected TTP can significantly improve the prognosis of mothers and fetus.

Keywords

• pregnancy
• thrombotic thrombocytopenic purpura
• plasma exchange
• steroids