Objective:

Approximately 1 in 25,000 to 1 in 100,000 pregnancies worldwide is complicated by TTP. It is characterized by hemolytic anemia, thrombocytopenia, and multi-vascular thrombi formation.Associated with high maternal and perinatal mortality, TTP can cause acute renal insufficiency, ischemic heart disease, altered mental status and end organ damage in mother and can lead to miscarriage and intrauterine fetal death.

We are presenting here a case of mid-trimester pregnancy which showed all the complications, however, she discharged home with her baby in good condition.

Case report:

A lady in 30's, gravida 2, para 1, with no living child, with severe thrombocytopenia at 24 weeks of gestation.

her first pregnancy was ended catastrophically at 24 weeks of gestation, she had fever, body ache, joint pain, hematuria, subconjunctival hemorrhage, and epistaxis, with high blood pressure, and very low platelet, and intrauterine fetal demise, managed in the intensive care unit, not investigated.

This pregnancy was unplanned, where she started to have high blood pressure with proteinuria, at 24 weeks, admitted and started on the antihypertensive medication, where she founds to have low platelet, for which she suspected to have Thrombotic thrombocytopenic purpura TTP, started on plasmapheresis and steroids, managed by multidisciplinary team, her condition deteriorated rapidly, stabilized and delivered by caesarian section at 26 weeks gestation, outcome A live baby girl weighing 730g.

Her final diagnosis was severe preeclampsia with HELLP syndrome, however, TTP could not be ruled out and ADAMTS-13 (confirmatory test) was still pending at the time of discharge. Patient discharged home in good condition on day 4 of delivery, on oral labetalol 200 mg twice per day, oral prednisolone, and Clexane for thromboprophylaxis.

Discussion:

Our patient had a good outcome in comparison to those reported in the literature.  She had presented with features of TTP in her first pregnancy and had a bad perinatal outcome, which went unrecognized. In the present pregnancy, she manifested the features of TTP at around the same weeks of gestation. Hereditary TTP occurring first time in pregnancy has a high rate of stillbirth (approximately 70%), especially if it occurs before 28 weeks of gestation.

Conclusion:

If we had failed to recognize TTP along with the superimposed pre-eclampsia in this pregnancy, she would have had a catastrophic result.

The presence of the multidisciplinary team with intensive care unit and neonatal care unit, added to the good outcome of this patient and her baby.