Frequent recurrence of pregnancy-triggered congenital thrombotic thrombocytopenic purpura: A rare case report

作者：Xu, Jialiang; Yu, Sijian; Zhang, Fuhua^*

来源：Clinical Hemorheology and Microcirculation, 2021, 77(2): 195-200.

DOI：10.3233/CH-200970

• Thrombotic thrombocytopenic purpura
• ADAMTS13
• pregnancy
• genetic mutations
• CSA

Thrombotic thrombocytopenic purpura (TTP) in adults is rare thrombotic microangiopathy (TMA), which is closely related to the lack of specific proteases of von Willebrand factor (vWF) multimers. It is currently believed that both congenital TTP (cTTP) and acquired TTP (aTTP) can induce acute attack through pregnancy. We report a case of a 24-yearold woman who was diagnosed as TTP during early pregnancy. A novel mutation was detected: c.3667G>T (p.E1223*). She responded well to plasma therapy during pregnancy and had a child by cesarean section at 32 weeks. TTP is still recurrent in postpartum patients. The plasma transfusion was effective, but caused a severe transfusion reaction. Cyclosporine was administered with the consent of the patient. This case showed cyclosporine-A (CSA) had a positive effect on ADAMTS13 activity. At 11-months follow-up, the patient's blood cells and LDH status were stable and no symptom was seen. Our case suggests that the patient had an unreported genetic mutation that causes TTP, immune factors may be involved in the onset of cTTP during pregnancy, and the use of immunosuppressive agents is effective in preventing recurrence.

• 单位
  南方医科大学