A rare case of thrombotic thrombocytopenic purpura during pregnancy with a successful outcome despite ovarian hyperstimulation syndrome during treatment

Abstract

Thrombotic microangiopathies during pregnancy are rare but may be life-threatening diseases for both the mother and the fetus. Thrombotic thrombocytopenic purpura (TTP) patients present with hemolytic anemia and thrombocytopenia associated with low ADAMTS-13 activity, a cleaving von Willebrand factor protein. Pregnancy has been described not only as a trigger of TTP but also as a phenotype modifier. In addition, hormonal changes induced by assisted reproduction technology (ART) swift the thrombotic – thrombolytic state towards coagulation, while increasing the pregnancy complications’ rate. We present a case of a 34-year-old pregnant woman, who conceived via ART and diagnosed with TTP at 13 weeks of gestation. She was initially treated with corticosteroids and daily plasmapheresis. Due to gradual unresponsiveness to treatment, rituximab was also added. After 3 doses, she was diagnosed with mild ovarian hyperstimulation syndrome, which resolved spontaneously after several weeks. She delivered a healthy neonate at 39+1 weeks via emergency cesarean section due to fetal distress, while the postpartum follow-up was uneventful. In conclusion, TTP should be suspected to a pregnant woman with raised hemorrhagic risk presenting with hematuria, anemia and thrombocytopenia.

Keywords

• Thrombotic thrombocytopenic purpura
• ART
• Pregnancy
• Rituximab
• ADAMTS-13
• OHSS