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Thrombotic thrombocytopenic purpura (TTP) is a rare, acute disorder of systemic microvascular thrombosis (incidence: 2cases/106/year). Most patients are women, many of reproductive age. Pregnancy is a recognized risk factor for triggering acute episodes. However pregnancy outcomes following recovery have been described in only six case reports. Therefore, we aimed to determine maternal and perinatal outcomes following recovery from TTP.
The Oklahoma TTP Registry is a prospective, population based inception cohort. Diagnosis of TTP was confirmed by severe ADAMTS13 deficiency (activity <10%). Pregnancy outcomes following recovery from TTP were documented by face-to-face interviews and medical records. Major complications were defined as TTP recurrence, pre-viable fetal loss, delivery <34 weeks, severe pre-eclampsia, other serious maternal medical complications, and NICU admission.
74 patients had severe acquired ADAMTS13 deficiency, 1995-2013; 57 (77%) were women; 46 (81%) survived their initial episode; 10 have had 15 subsequent pregnancies (Table). 12 (80%) pregnancies resulted in live born infants who have become healthy children. 5 women had major complications in 6 pregnancies; none died or had significant sequelae. The major complications were: 2 women (2 pregnancies) had recurrent TTP post-partum; 1 woman also had severe preeclampsia; both recovered with appropriate treatment. 1 woman developed severe pre-eclampsia in both of her pregnancies. 1 woman had a 20 week fetal loss. 1 woman had a severe lupus flare. 3 infants (25%) required NICU admission; 2 were related to the timing of elective delivery, not to a pregnancy complication.
Pregnancy following recovery from TTP may have increased risk for complications, including recurrent TTP. However these risks are manageable and 80% of pregnancies resulted in healthy children. We conclude that these women should not be discouraged from future pregnancies.
1Patients and pregnancies after TTP recovery