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
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...
| Main Authors: | , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2022-06-01
|
| Series: | Diagnostics |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2075-4418/12/7/1559 |
| _version_ | 1827597544828436480 |
|---|---|
| author | Melinda Ildiko Mitranovici Lucian Pușcașiu Ioan Emilian Oală Izabella Petre Marius Lucian Craina Antonia Rebeka Mager Kinga Vasile Diana Maria Chiorean Adrian-Horațiu Sabău Sabin Gligore Turdean Ovidiu Simion Cotoi |
| author_facet | Melinda Ildiko Mitranovici Lucian Pușcașiu Ioan Emilian Oală Izabella Petre Marius Lucian Craina Antonia Rebeka Mager Kinga Vasile Diana Maria Chiorean Adrian-Horațiu Sabău Sabin Gligore Turdean Ovidiu Simion Cotoi |
| author_sort | Melinda Ildiko Mitranovici |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-09T03:32:48Z |
| format | Article |
| id | doaj.art-ca976c6714514023a53d68bea85b784c |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-09T03:32:48Z |
| publishDate | 2022-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-ca976c6714514023a53d68bea85b784c2023-12-03T14:53:38ZengMDPI AGDiagnostics2075-44182022-06-01127155910.3390/diagnostics12071559A 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 PregnancyMelinda Ildiko Mitranovici0Lucian Pușcașiu1Ioan Emilian Oală2Izabella Petre3Marius Lucian Craina4Antonia Rebeka Mager5Kinga Vasile6Diana Maria Chiorean7Adrian-Horațiu Sabău8Sabin Gligore Turdean9Ovidiu Simion Cotoi10Department of Obstetrics and Gynecology, Emergency County Hospital Hunedoara, 14 Victoriei Street, 331057 Hunedoara, RomaniaDepartment of Obstetrics and Gynecology, County Emergency Hospital, University of Medicine and Pharmacy Targu Mures, 38 Gh. Marinescu Str., 540142 Targu Mures, RomaniaDepartment of Obstetrics and Gynecology, Emergency County Hospital Hunedoara, 14 Victoriei Street, 331057 Hunedoara, RomaniaDepartment of Obstetrics and Gynecology, Victor Babeș University of Medicine and Pharmacy, 2 Eftimie Murgu Sq., 300041 Timisoara, RomaniaDepartment of Obstetrics and Gynecology, Victor Babeș University of Medicine and Pharmacy, 2 Eftimie Murgu Sq., 300041 Timisoara, RomaniaDepartment of Pathology, Emergency County Hospital Hunedoara, 14 Victoriei Street, 331057 Hunedoara, RomaniaDepartment of Hematology, Emergency County Hospital Deva, 1 Decembrie Street, 330005 Deva, RomaniaDepartment of Pathology, County Clinical Hospital of Targu Mures, 540072 Targu Mures, RomaniaDepartment of Pathology, County Clinical Hospital of Targu Mures, 540072 Targu Mures, RomaniaDepartment of Pathology, County Clinical Hospital of Targu Mures, 540072 Targu Mures, RomaniaDepartment of Pathology, County Clinical Hospital of Targu Mures, 540072 Targu Mures, RomaniaThrombocytopenic 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.https://www.mdpi.com/2075-4418/12/7/1559thrombotic thrombocytopenic purpuravon Willebrand factor proteaseADAMTS13vascular microthrombosispregnancyfetal–maternal mortality |
| spellingShingle | Melinda Ildiko Mitranovici Lucian Pușcașiu Ioan Emilian Oală Izabella Petre Marius Lucian Craina Antonia Rebeka Mager Kinga Vasile Diana Maria Chiorean Adrian-Horațiu Sabău Sabin Gligore Turdean Ovidiu Simion Cotoi 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 Diagnostics thrombotic thrombocytopenic purpura von Willebrand factor protease ADAMTS13 vascular microthrombosis pregnancy fetal–maternal mortality |
| title | 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 |
| title_full | 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 |
| title_fullStr | 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 |
| title_full_unstemmed | 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 |
| title_short | 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 |
| title_sort | 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 |
| topic | thrombotic thrombocytopenic purpura von Willebrand factor protease ADAMTS13 vascular microthrombosis pregnancy fetal–maternal mortality |
| url | https://www.mdpi.com/2075-4418/12/7/1559 |
| work_keys_str_mv | AT melindaildikomitranovici araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT lucianpuscasiu araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT ioanemilianoala araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT izabellapetre araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT mariusluciancraina araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT antoniarebekamager araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT kingavasile araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT dianamariachiorean araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT adrianhoratiusabau araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT sabingligoreturdean araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT ovidiusimioncotoi araceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT melindaildikomitranovici raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT lucianpuscasiu raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT ioanemilianoala raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT izabellapetre raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT mariusluciancraina raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT antoniarebekamager raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT kingavasile raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT dianamariachiorean raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT adrianhoratiusabau raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT sabingligoreturdean raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy AT ovidiusimioncotoi raceagainsttheclockacasereportandliteraturereviewconcerningtheimportanceofadamts13testingindiagnosisandmanagementofthromboticthrombocytopenicpurpuraduringpregnancy |