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Kidney Week

Abstract: PO0303

Pregnancy and Hereditary Thrombotic Thrombocytopenic Purpura: A Case Report

Session Information

Category: Acute Kidney Injury

  • 101 AKI: Epidemiology, Risk Factors, and Prevention

Authors

  • Abdel Massih, Sarah, Virginia Commonwealth University Health System, Richmond, Virginia, United States
  • Kidd, Jason M., Virginia Commonwealth University Health System, Richmond, Virginia, United States
Introduction

Pregnancy and postpartum have been recognized as periods of high risk for development of thrombotic microangiopathy (TMA). We report a case of hereditary thrombotic thrombocytopenic purpura (TTP) unmasked by pregnancy.

Case Description

A previously healthy 32-year-old Caucasian female G1P1 24 weeks pregnant presented with urinary frequency, dark urine and diarrhea for 1-2 weeks. On initial presentation, blood pressure was at 153/96 mmHg. Labs were significant for a rapidly dropping Hgb with hemolysis, thrombocytopenia with a platelet count of 11, mild transaminitis, and a rising serum creatinine with no antecedent history of renal disease (table 1).
Unfortunately, an ultrasound revealed intrauterine fetal demise. She received 2 units of platelets and underwent an uneventful delivery. Following delivery, all parameters including Hgb, platelets and Cr improved (table 1). Two days post-delivery, ADAMTS-13 level returned at <2%. She was started on high dose prednisone and received 4 sessions of plasmapheresis. ADAMTS-13 antibody was later found to be negative.

Discussion

TTP is caused by deficiency of ADAMTS-13 metalloproteinase which cleaves the Von Willebrand factor. Low activity of ADAMTS-13 (20-40%) is seen in patient with preeclampsia, eclampsia, HELLP syndrome and pregnancy associated hemolytic uremic syndrome. The enzyme level will decrease to less than 20% only in pregnancy associated TTP.
Though TTP in the general population is mostly immune in nature, 24% of cases of pregnancy associated TTP are hereditary. Delivery has been shown to achieve rapid remission in many cases. Most authorities recommend initiation of plasmapheresis with TMA and pregnancy when platelet count is less than 30 with later addition of steroids when the diagnosis of TTP is confirmed. The response rate to treatment is 80-90%.
Immune TTP may reoccur in future pregnancies but hereditary TTP has a 100% risk of relapse with future pregnancies making it essential to differentiate the two entities for appropriate management of future conceptions which requires plasma exchange starting early in pregnancy.

Table 1
 Pre-deliveryPost-deliveryPost-plasmapheresis
Hb (mg/dL)13.9-->6.98.211.2
Platelets x10^9/L1169521
LDH (U/L)1616  
Haptoglobin (mg/dL)<8 238
Cr (mg/dL)2.5-->3.72.651