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.


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.

Conflict(s) of Interest

All authors have no conflicts of interest to disclose.

References with DOI

1. McMinn JR, George JN. Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy. J clin apheresis. 2001;16(4):202-9. https://doi.org/10.1002/jca.10005

2. Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol. 1982;142(2):159-67. https://doi.org/10.1016/j.ajog.2005.02.113

3. Fyfe-Brown A, Clarke G, Nerenberg K, Chandra S, Jain V. Management of pregnancy-associated thrombotic thrombocytopenia purpura. AJP reports. 2013;3(1):45-50. https://doi.org/10.1055/s-0032-1331380

4. Lucania G, Camiolo E, Carmina MG, Fiandaca T, Indovina A, Malato A, et al. Multidisciplinary approach in pregnancy-associated thrombotic thrombocytopenic purpura: a case report. Blood transfusion = Trasfusione del sangue. 2014;12 Suppl 1:s137-40.

5. Falter T, Kremer Hovinga JA, Lackner K, Fullemann HG, Lammle B, Scharrer I. Late onset and pregnancy-induced congenital thrombotic thrombocytopenic purpura. Hamostaseologie. 2014;34(3):244-8. https://doi.org/10.5482/hamo-14-03-0023

6. George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654-66. https://doi.org/10.1056/nejmra1312353

7. Fujimura Y, Matsumoto M, Kokame K, Isonishi A, Soejima K, Akiyama N, et al. Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients. Br J Haematol. 2009;144(5):742-54. https://doi.org/10.1111/j.1365-2141.2008.07515.x

8. Scully M, Thomas M, Underwood M, Watson H, Langley K, Camilleri RS, et al. Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes. Blood. 2014;124(2):211-9. https://doi.org/10.1182/blood-2014-02-553131

9. George JN. The association of pregnancy with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Curr Opin Hematol. 2003;10(5):339-44. ttps://doi.org/10.1097/00062752-200309000-00003

10. Jiang Y, McIntosh JJ, Reese JA, Deford CC, Kremer Hovinga JA, Lammle B, et al. Pregnancy outcomes following recovery from acquired thrombotic thrombocytopenic purpura. Blood. 2014;123(11):1674-80. https://doi.org/10.1182/blood-2013-11-538900

11. Rosove MH. Thrombotic microangiopathies. Semin Arthritis Rheum. 2014;43(6):797-805.

Creative Commons License

Creative Commons Attribution 4.0 License
This work is licensed under a Creative Commons Attribution 4.0 License.