Management of Evans’ Syndrome in Pregnancy: A Case Report and a Narrative Review
<b>Background/Objectives</b>: Evans’ syndrome (ES) is a rare autoimmune disorder characterized by the simultaneous or sequential onset of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Given its rarity, available data concerning the clinical course and optimal mana...
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MDPI AG
2025-03-01
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| Series: | Reproductive Medicine |
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| Online Access: | https://www.mdpi.com/2673-3897/6/1/6 |
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| author | Giulia Fontana Micaela Fredi Cecilia Nalli Rossana Orabona Brunetta Guaragni Laura Picciau Valeria Cancelli Laura Andreoli Sonia Zatti Angela Tincani |
| author_facet | Giulia Fontana Micaela Fredi Cecilia Nalli Rossana Orabona Brunetta Guaragni Laura Picciau Valeria Cancelli Laura Andreoli Sonia Zatti Angela Tincani |
| author_sort | Giulia Fontana |
| collection | DOAJ |
| description | <b>Background/Objectives</b>: Evans’ syndrome (ES) is a rare autoimmune disorder characterized by the simultaneous or sequential onset of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Given its rarity, available data concerning the clinical course and optimal management in pregnancy are limited. <b>Methods</b>: We present the case of a 32-year-old woman who experienced ES during her first pregnancy. She had been previously diagnosed with childhood-onset SLE at the age of 14 but had been in treatment-free remission since the age of 24. The treatment of both AIHA and ITP included intravenous immunoglobulins, cyclosporine-A, high dosage oral corticosteroids, and, in the second trimester, rituximab. The delivery was planned at 34 + 6 weeks of gestation (GW); no immunological alterations or infectious complications were detected in the newborn. The post-delivery period was uncomplicated, and the mother was discharged with a normal blood count. A narrative review of available ES cases during pregnancy is also presented. <b>Results</b>: A total of 16 patients with ES in pregnancy were reported, including the one we described. None of them developed major bleedings during gestation, while a case of abruptio placentae with delayed postpartum hemorrhage occurred. ITP was difficult to treat in 4/16 women after delivery; 4/16 patients also developed gestational hypertensive disorders. Perinatal outcomes include 13/17 healthy newborns and 4/17 stillbirths (2 of them were twins), of which 1 was due to fetal AIHA. Gestational age at birth was before 37 GW in 8/17. In 15/16 women, ES resolved after delivery. <b>Conclusions</b>: The occurrence of ES during pregnancy has been rarely reported; it constitutes a clinical challenge due to the need for multiple treatments, including conventional immunosuppressants and/or biologic drugs as steroid-sparing agents. After delivery, ES appeared to be less resistant to treatment than it was during pregnancy. |
| format | Article |
| id | doaj-art-43647694df3242959e888b582f0ff081 |
| institution | OA Journals |
| issn | 2673-3897 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Reproductive Medicine |
| spelling | doaj-art-43647694df3242959e888b582f0ff0812025-08-20T01:48:58ZengMDPI AGReproductive Medicine2673-38972025-03-0161610.3390/reprodmed6010006Management of Evans’ Syndrome in Pregnancy: A Case Report and a Narrative ReviewGiulia Fontana0Micaela Fredi1Cecilia Nalli2Rossana Orabona3Brunetta Guaragni4Laura Picciau5Valeria Cancelli6Laura Andreoli7Sonia Zatti8Angela Tincani9Rheumatology and Clinical Immunology Unit and Clinical and Experimental Sciences Department, ERN ReCONNET Centre, ASST Spedali Civili, University of Brescia, 25123 Brescia, ItalyRheumatology and Clinical Immunology Unit and Clinical and Experimental Sciences Department, ERN ReCONNET Centre, ASST Spedali Civili, University of Brescia, 25123 Brescia, ItalyRheumatology and Clinical Immunology Unit and Clinical and Experimental Sciences Department, ERN ReCONNET Centre, ASST Spedali Civili, University of Brescia, 25123 Brescia, ItalyObstetrics and Gynecology Unit and Clinical, Experimental Sciences Department, ASST Spedali Civili, University of Brescia, 25123 Brescia, ItalyNeonatal Intensive Care Unit, Children’s Hospital, ASST Spedali Civili, 25123 Brescia, ItalyNeonatal Intensive Care Unit, Children’s Hospital, ASST Spedali Civili, 25123 Brescia, ItalyDivision of Hematology, ASST Spedali Civili, 25123 Brescia, ItalyRheumatology and Clinical Immunology Unit and Clinical and Experimental Sciences Department, ERN ReCONNET Centre, ASST Spedali Civili, University of Brescia, 25123 Brescia, ItalyObstetrics and Gynecology Unit and Clinical, Experimental Sciences Department, ASST Spedali Civili, University of Brescia, 25123 Brescia, ItalyRheumatology and Clinical Immunology Unit and Clinical and Experimental Sciences Department, ERN ReCONNET Centre, ASST Spedali Civili, University of Brescia, 25123 Brescia, Italy<b>Background/Objectives</b>: Evans’ syndrome (ES) is a rare autoimmune disorder characterized by the simultaneous or sequential onset of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Given its rarity, available data concerning the clinical course and optimal management in pregnancy are limited. <b>Methods</b>: We present the case of a 32-year-old woman who experienced ES during her first pregnancy. She had been previously diagnosed with childhood-onset SLE at the age of 14 but had been in treatment-free remission since the age of 24. The treatment of both AIHA and ITP included intravenous immunoglobulins, cyclosporine-A, high dosage oral corticosteroids, and, in the second trimester, rituximab. The delivery was planned at 34 + 6 weeks of gestation (GW); no immunological alterations or infectious complications were detected in the newborn. The post-delivery period was uncomplicated, and the mother was discharged with a normal blood count. A narrative review of available ES cases during pregnancy is also presented. <b>Results</b>: A total of 16 patients with ES in pregnancy were reported, including the one we described. None of them developed major bleedings during gestation, while a case of abruptio placentae with delayed postpartum hemorrhage occurred. ITP was difficult to treat in 4/16 women after delivery; 4/16 patients also developed gestational hypertensive disorders. Perinatal outcomes include 13/17 healthy newborns and 4/17 stillbirths (2 of them were twins), of which 1 was due to fetal AIHA. Gestational age at birth was before 37 GW in 8/17. In 15/16 women, ES resolved after delivery. <b>Conclusions</b>: The occurrence of ES during pregnancy has been rarely reported; it constitutes a clinical challenge due to the need for multiple treatments, including conventional immunosuppressants and/or biologic drugs as steroid-sparing agents. After delivery, ES appeared to be less resistant to treatment than it was during pregnancy.https://www.mdpi.com/2673-3897/6/1/6Evans’ syndromepregnancyoutcomesrituximab |
| spellingShingle | Giulia Fontana Micaela Fredi Cecilia Nalli Rossana Orabona Brunetta Guaragni Laura Picciau Valeria Cancelli Laura Andreoli Sonia Zatti Angela Tincani Management of Evans’ Syndrome in Pregnancy: A Case Report and a Narrative Review Reproductive Medicine Evans’ syndrome pregnancy outcomes rituximab |
| title | Management of Evans’ Syndrome in Pregnancy: A Case Report and a Narrative Review |
| title_full | Management of Evans’ Syndrome in Pregnancy: A Case Report and a Narrative Review |
| title_fullStr | Management of Evans’ Syndrome in Pregnancy: A Case Report and a Narrative Review |
| title_full_unstemmed | Management of Evans’ Syndrome in Pregnancy: A Case Report and a Narrative Review |
| title_short | Management of Evans’ Syndrome in Pregnancy: A Case Report and a Narrative Review |
| title_sort | management of evans syndrome in pregnancy a case report and a narrative review |
| topic | Evans’ syndrome pregnancy outcomes rituximab |
| url | https://www.mdpi.com/2673-3897/6/1/6 |
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