Rare Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting as Posttransplant Thrombotic Microangiopathy
Middle-aged female underwent transplantation with her mother as a donor after appropriate desensitization. The patient developed graft dysfunction with systemic features of thrombotic microangiopathy. Renal allograft biopsy was suggestive of antibody-mediated rejection. She was treated with plasmaph...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2024-12-01
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| Series: | Indian Journal of Transplantation |
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| Online Access: | https://journals.lww.com/10.4103/ijot.ijot_29_24 |
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| author | Akila Viswanathan Vidyashankar Panchangam Shashank Shetty Govardhan Reddy Sanjeev S. Tonshal Prakash Doraiswamy M. R. Shashank Kiran Krishne Gowda Parameswaran Anoop |
| author_facet | Akila Viswanathan Vidyashankar Panchangam Shashank Shetty Govardhan Reddy Sanjeev S. Tonshal Prakash Doraiswamy M. R. Shashank Kiran Krishne Gowda Parameswaran Anoop |
| author_sort | Akila Viswanathan |
| collection | DOAJ |
| description | Middle-aged female underwent transplantation with her mother as a donor after appropriate desensitization. The patient developed graft dysfunction with systemic features of thrombotic microangiopathy. Renal allograft biopsy was suggestive of antibody-mediated rejection. She was treated with plasmapheresis followed by intravenous immunoglobulin. However, due to lack of response, further workup was sent and the patient was found to have ADAMTS 13 levels <1% with no evidence of antibodies. Hence, she was diagnosed to have congenital thrombotic thrombocytopenic purpura. A graft nephrectomy was done, and the patient was managed with frozen plasma infusions until remission. Currently, the patient is back on maintenance hemodialysis and is doing well and planned for a genetic study. |
| format | Article |
| id | doaj-art-7fc69dfd026346ba8d0833360e9acd3d |
| institution | Kabale University |
| issn | 2212-0017 2212-0025 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Transplantation |
| spelling | doaj-art-7fc69dfd026346ba8d0833360e9acd3d2025-01-07T06:12:53ZengWolters Kluwer Medknow PublicationsIndian Journal of Transplantation2212-00172212-00252024-12-0118445345510.4103/ijot.ijot_29_24Rare Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting as Posttransplant Thrombotic MicroangiopathyAkila ViswanathanVidyashankar PanchangamShashank ShettyGovardhan ReddySanjeev S. TonshalPrakash DoraiswamyM. R. ShashankKiran Krishne GowdaParameswaran AnoopMiddle-aged female underwent transplantation with her mother as a donor after appropriate desensitization. The patient developed graft dysfunction with systemic features of thrombotic microangiopathy. Renal allograft biopsy was suggestive of antibody-mediated rejection. She was treated with plasmapheresis followed by intravenous immunoglobulin. However, due to lack of response, further workup was sent and the patient was found to have ADAMTS 13 levels <1% with no evidence of antibodies. Hence, she was diagnosed to have congenital thrombotic thrombocytopenic purpura. A graft nephrectomy was done, and the patient was managed with frozen plasma infusions until remission. Currently, the patient is back on maintenance hemodialysis and is doing well and planned for a genetic study.https://journals.lww.com/10.4103/ijot.ijot_29_24plasmapheresisposttransplantthrombotic microangiopathy |
| spellingShingle | Akila Viswanathan Vidyashankar Panchangam Shashank Shetty Govardhan Reddy Sanjeev S. Tonshal Prakash Doraiswamy M. R. Shashank Kiran Krishne Gowda Parameswaran Anoop Rare Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting as Posttransplant Thrombotic Microangiopathy Indian Journal of Transplantation plasmapheresis posttransplant thrombotic microangiopathy |
| title | Rare Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting as Posttransplant Thrombotic Microangiopathy |
| title_full | Rare Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting as Posttransplant Thrombotic Microangiopathy |
| title_fullStr | Rare Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting as Posttransplant Thrombotic Microangiopathy |
| title_full_unstemmed | Rare Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting as Posttransplant Thrombotic Microangiopathy |
| title_short | Rare Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting as Posttransplant Thrombotic Microangiopathy |
| title_sort | rare case of congenital thrombotic thrombocytopenic purpura presenting as posttransplant thrombotic microangiopathy |
| topic | plasmapheresis posttransplant thrombotic microangiopathy |
| url | https://journals.lww.com/10.4103/ijot.ijot_29_24 |
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