Post-splenectomy Posterior Reversible Encephalopathy Syndrome in a β-Thalassemia Major child with Evans Syndrome
Patients with β-Thalassemia Major are at risk of alloimmunization and autoimmunization because they need regular multiple blood transfusions. Here we are reporting a case of an 8 years old male child, known case of β-thalassemia major, who developed autoimmune pancytopenia, known as Evans syndrome,...
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| Format: | Article |
| Language: | English |
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Gujarat Adani Institute of Medical Sciences
2023-06-01
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| Series: | GAIMS Journal of Medical Sciences |
| Subjects: | |
| Online Access: | https://gjms.gaims.ac.in/index.php/gjms/article/view/77/80 |
| _version_ | 1797805682077990912 |
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| author | Rekha Thaddanee Ekta Thacker Shamim Morbiwala Vinisha Makhijani Bhushan M Warpe Vishva Sureja |
| author_facet | Rekha Thaddanee Ekta Thacker Shamim Morbiwala Vinisha Makhijani Bhushan M Warpe Vishva Sureja |
| author_sort | Rekha Thaddanee |
| collection | DOAJ |
| description | Patients with β-Thalassemia Major are at risk of alloimmunization and autoimmunization because they need regular multiple blood transfusions. Here we are reporting a case of an 8 years old male child, known case of β-thalassemia major, who developed autoimmune pancytopenia, known as Evans syndrome, and post-splenectomy neurological complication Posterior Reversible Encephalopathy Syndrome (PRES). Evans syndrome (ES) is a rare autoimmune disorder characterized by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. There is no established evidence-based treatment. Steroids are used as a first-line therapy. Intravenous immunoglobulin is used as a life-saving therapy in severe cases. Rituximab and splenectomy are used as a second-line therapy. PRES is linked with hypertension. Characteristic clinical features of PRES are headache, blurring of vision, seizures, altered consciousness, cortical blindness or transient motor deficit. |
| first_indexed | 2024-03-13T05:55:45Z |
| format | Article |
| id | doaj.art-3538f5348d2d4c489d7fe622ca7e12dc |
| institution | Directory Open Access Journal |
| issn | 2583-1763 |
| language | English |
| last_indexed | 2024-03-13T05:55:45Z |
| publishDate | 2023-06-01 |
| publisher | Gujarat Adani Institute of Medical Sciences |
| record_format | Article |
| series | GAIMS Journal of Medical Sciences |
| spelling | doaj.art-3538f5348d2d4c489d7fe622ca7e12dc2023-06-13T05:11:51ZengGujarat Adani Institute of Medical SciencesGAIMS Journal of Medical Sciences2583-17632023-06-0132293310.5281/zenodo.8031301Post-splenectomy Posterior Reversible Encephalopathy Syndrome in a β-Thalassemia Major child with Evans SyndromeRekha Thaddanee0https://orcid.org/0000-0001-9503-6388Ekta Thacker1Shamim Morbiwala2Vinisha Makhijani3Bhushan M Warpe4Vishva Sureja5Department of Pediatrics, Gujarat Adani Institute of Medical Sciences and GK General Hospital, Bhuj, Kachchh, Gujarat-370001Department of Pediatrics, Gujarat Adani Institute of Medical Sciences and GK General Hospital, Bhuj, Kachchh, Gujarat-370001Department of Pediatrics, Gujarat Adani Institute of Medical Sciences and GK General Hospital, Bhuj, Kachchh, Gujarat-370001Department of Pediatrics, Gujarat Adani Institute of Medical Sciences and GK General Hospital, Bhuj, Kachchh, Gujarat-370001Department of Pathology, Gujarat Adani Institute of Medical Sciences and GK General Hospital, Bhuj, Kachchh, Gujarat-370001Department of Pathology, Gujarat Adani Institute of Medical Sciences and GK General Hospital, Bhuj, Kachchh, Gujarat-370001Patients with β-Thalassemia Major are at risk of alloimmunization and autoimmunization because they need regular multiple blood transfusions. Here we are reporting a case of an 8 years old male child, known case of β-thalassemia major, who developed autoimmune pancytopenia, known as Evans syndrome, and post-splenectomy neurological complication Posterior Reversible Encephalopathy Syndrome (PRES). Evans syndrome (ES) is a rare autoimmune disorder characterized by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. There is no established evidence-based treatment. Steroids are used as a first-line therapy. Intravenous immunoglobulin is used as a life-saving therapy in severe cases. Rituximab and splenectomy are used as a second-line therapy. PRES is linked with hypertension. Characteristic clinical features of PRES are headache, blurring of vision, seizures, altered consciousness, cortical blindness or transient motor deficit.https://gjms.gaims.ac.in/index.php/gjms/article/view/77/80autoimmune hemolytic anemiaautoimmune thrombocytopeniaautoimmune neutropeniaevans syndromeposterior reversible encephalopathy syndrome |
| spellingShingle | Rekha Thaddanee Ekta Thacker Shamim Morbiwala Vinisha Makhijani Bhushan M Warpe Vishva Sureja Post-splenectomy Posterior Reversible Encephalopathy Syndrome in a β-Thalassemia Major child with Evans Syndrome GAIMS Journal of Medical Sciences autoimmune hemolytic anemia autoimmune thrombocytopenia autoimmune neutropenia evans syndrome posterior reversible encephalopathy syndrome |
| title | Post-splenectomy Posterior Reversible Encephalopathy Syndrome in a β-Thalassemia Major child with Evans Syndrome |
| title_full | Post-splenectomy Posterior Reversible Encephalopathy Syndrome in a β-Thalassemia Major child with Evans Syndrome |
| title_fullStr | Post-splenectomy Posterior Reversible Encephalopathy Syndrome in a β-Thalassemia Major child with Evans Syndrome |
| title_full_unstemmed | Post-splenectomy Posterior Reversible Encephalopathy Syndrome in a β-Thalassemia Major child with Evans Syndrome |
| title_short | Post-splenectomy Posterior Reversible Encephalopathy Syndrome in a β-Thalassemia Major child with Evans Syndrome |
| title_sort | post splenectomy posterior reversible encephalopathy syndrome in a β thalassemia major child with evans syndrome |
| topic | autoimmune hemolytic anemia autoimmune thrombocytopenia autoimmune neutropenia evans syndrome posterior reversible encephalopathy syndrome |
| url | https://gjms.gaims.ac.in/index.php/gjms/article/view/77/80 |
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