Safety and efficacy of caplacizumab in a case of thrombotic thrombocytopenic purpura in the postpartum period

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening disease for which pregnancy and the postpartum period represent risk factors. Here, we present the case of a 39-year-old woman at the 31st week of gestation, who presented with cutaneous haemorrhagic symptoms. The complete blood count showed anaemia, thrombocytopenia, increase in haemolysis indices and undetectable ADAMTS13 activity. Acquired TTP was diagnosed, and she started daily plasma exchange (PEX) and methylprednisolone. After 5 days, an emergency caesarean section was performed with success because of pathologic cardiotocographic findings. After 7 days of PEX, the patient showed an initial laboratoristic improvement; unfortunately, 3 days later, she had a recurrence of disease and started daily PEX, caplacizumab and steroid, obtaining a haematological improvement. No literature data about caplacizumab use in pregnant or breastfeeding patients are available. In the present study, we describe that caplacizumab in the postpartum period could be well tolerated and effective.