Keywords
TTP, Congenital TTP, Pregnancy, HELLP, Preeclampsia, Upshaw-Schulman syndrome
Disciplines
Medicine and Health Sciences
Abstract
Introduction: Thrombotic thrombocytopenic purpura (TTP) in pregnancy is rare and can be fatal if misdiagnosed.Typically patients present with microangiopathic hemolytic anemia, thrombocytopenia, Altered mental status, fever, and renal abnormalities
Case Description:
We are presenting a case of 26 year old pregnant female presented with elevated BP. She had scattered Bruises in her legs, thighs and arms along with +2 bilateral pitting leg edema. Initial and subsequent laboratory studies showed features of microangiopathic hemolytic anemia (MAHA), which brought up the suspicion of HELLP (hemolysis, elevated liver enzymes, low platelets )syndrome initially. Labor was induced after transfusion of 2 units of PRBCs and 2 units of Platelets. The patient platelets and hemoglobin showed no improvement after delivery and she continued to have hemolysis despite delivery and steroids administration. So daily therapeutic plasma exchange was initiated as the index of suspicion for TTP increased. After two sessions, there was a significant improvement in the platelet count and hemolysis profile which were normalized by the 4th session.
Conclusion:
Congenital TTP is a rare syndrome that clinicians should be aware of. Early initiation of plasma exchange dramatically improves survival from less than 10% to approximately 80%. This can only be achieved by having high level of suspicion and awareness to this possibility.
