A Race against the Clock: A Case Report and Literature Review Concerning the Importance of ADAMTS13 Testing in Diagnosis and Management of Thrombotic Thrombocytopenic Purpura during Pregnancy
Open Access
- 27 June 2022
- journal article
- case report
- Published by MDPI AG in Diagnostics
- Vol. 12 (7), 1559
- https://doi.org/10.3390/diagnostics12071559
Abstract
Thrombocytopenic purpura (TTP) is a rare, potentially fatal pathology characterized by microangiopathic thrombotic syndrome and caused by an acute protease deficiency of von Willebrand factor, ADAMTS13. Moreover, ADAMTS13 deficiency promotes microthrombosis led by the persistence of ultra-large VWF multimers in the blood circulation. According to the few studies involving pregnant participants, the heterogeneity of manifestations has made this pathology difficult to diagnose, with an unexpected occurrence and increased risk of maternal and fetal morbidity and mortality. We reported on the case of a 28-year-old pregnant woman with an obstetric score of G2P0 who presented to the obstetrics and gynecology department of our clinic with the complaint of minimal vaginal bleeding. The evolution of our case was severe and life-threatening, a “race against the clock”, with our goal being to emphasize the importance and difficulty of diagnosing TTP in the absence of specific symptomatology. We faced a lack of technological support for a correct and complete diagnosis, and the first manifestation of this disease was the intrauterine death of the fetus. After completing all the necessary procedures, the placental tissue was sent for further histopathological evaluation. We highlighted the importance of monitoring ADAMTS13 for relapses monthly, with prophylaxis being essential for maternal and fetal mortality and morbidity.This publication has 31 references indexed in Scilit:
- Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathyThe Lancet Haematology, 2016
- Pathology Consultation on the Diagnosis and Treatment of Thrombotic Microangiopathies (TMAs)American Journal of Clinical Pathology, 2016
- Thrombotic thrombocytopenic purpura during pregnancy versus imitator of preeclampsiaTransfusion, 2015
- Pregnancy complications in acquired thrombotic thrombocytopenic purpura: a case–control studyOrphanet Journal of Rare Diseases, 2014
- Multidisciplinary approach in pregnancy-associated thrombotic thrombocytopenic purpura: a case report2014
- The impact of congenital thrombotic thrombocytopenic purpura on pregnancy complicationsThrombosis and Haemostasis, 2014
- Laparoscopic splenectomy for the treatment of refractory thrombotic thrombocytopenic purpuraClinical Journal of Gastroenterology, 2013
- Unfavorable course in pregnancy-associated thrombotic thrombocytopenic purpura necessitating a perimortem Cesarean section: a case reportJournal of Medical Case Reports, 2013
- Delayed visual recovery in pregnancy-associated thrombotic thrombocytopenic purpura with bilateral serous retinal detachmentDocumenta Ophthalmologica, 2012
- Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpuraBlood, 2012