Objective

To explore the diagnosis and treatment of pregnancy complicated with thrombotic thrombocytopenic purpura （TTP）.

Methods

Two cases of pregnancy complicated with TTP admitted to the Third Affiliated Hospital of Guangzhou Medical University on September 3，2022，and February 17，2024（patient 1 and patient 2）were selected as the subjects of this study.A retrospective analysis of their clinical characteristics，laboratory test results，diagnosis and treatment processes，and outcomes was conducted.Additionally，literature related to TTP in pregnancy from domestic and international databases was searched to summarize the clinical manifestations，diagnosis，and treatment of pregnancy complicated with TTP.The procedures followed in this study complied with the requirements of the 2013 revision of the WorldMedicalAssociationDeclarationofHelsinki.

Results

①patient 1 was admitted due to"amenorrhea at 36⁺³ weeks，both lower limb edema for 2 weeks，and elevated blood pressure for 2 days"，with a history of systemic lupus erythematosus（SLE）.At 36⁺³ weeks of gestation，she developed severe decrease of platelet count（PLT）and hemolytic anemia，which led to the consideration of"lupus crisis"，then an emergency cesarean section was performed.According to the activity of ADAMTS13 was measured to be 0.1%，patient 1 was confirmed the diagnosis of SLE complicated with TTP.After platelet transfusion，red blood cell transfusion，plasma exchange，and corticosteroid therapy for patient 1，she was successfully treated.②Patient 2 was admitted due to"elevated liver enzymes and thrombocytopenia for 6⁺h after cesarean section".At 36⁺⁴ weeks of gestation，she underwent an emergency cesarean section due to severe preeclampsia （sPE）.Postoperatively，she experienced a decrease in PLT and an increase in liver enzymes，suspected to have hemolysis，elevated liver enzymes，low platelet count（HELLP）syndrome.Subsequently，the PLT further decreased，accompanied by h emolytic anemia.According to the activity and inhibitor of ADAMTS13 test results，a diagnosis of TTP was confirmed of patient 2.Patient 2 was discharged after plasma exchange，blood product transfusion，and blood purification therapy，and her renal function returned to normal after a 1-month follow-up.③A search conducted according to the literature search strategy set in this study showed a total of 97 pieces of literature about pregnancy complicated with TTP，including 125 cases of pregnancy complicated with TTP，and plus 2 cases （patient 1 and patient 2），there were 127 cases of pregnancy complicated with TTP.The analysis of case data of 127 cases showed that 63 cases （49.6%）presented with the TTP triad，and 8 cases （6.3%）presented with the TTP pentad；58 cases （45.7%）underwent ADAMTS13 activity testing，with 48 cases （82.8%）showing severe reduction in activity；100 cases （78.7%）underwent plasma exchange treatment，with 25 cases （25.0%）resulting in death，and among the 27 cases（21.3%）who did not receive plasma exchange treatment，12 cases （44.4%）resulting in death.

Conclusions

Pregnancy is one of the triggers for TTP in pregnant women，and pregnant women with clinical manifestations of microangiopathic hemolytic anemia （MAHA）and thrombocytopenia should be vigilant about this disease.ADAMTS13 activity t esting aids in the diagnosis of TTP.Plasma exchange is an effective treatment measure for pregnant women with TTP after delivery.