The Challenges of Distinguishing Different Causes of TMA in a Pregnant Kidney Transplant Recipient
Thrombotic microangiopathy (TMA) reflects a syndrome of endothelial injury characterised by microangiopathic haemolytic anaemia (nonimmune), thrombocytopenia, and often end-organ dysfunction. TMA disorders are well-recognised in kidney transplant recipients, often due to an underlying genetic predis...
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| Format: | Article |
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Wiley
2024-01-01
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| Series: | Case Reports in Nephrology |
| Online Access: | http://dx.doi.org/10.1155/2024/9218637 |
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| author | A. Krelle S. Price M. M. Law S. Kranz P. Shamdasani S. Kane J. Unterscheider P. Champion de Crespigny |
| author_facet | A. Krelle S. Price M. M. Law S. Kranz P. Shamdasani S. Kane J. Unterscheider P. Champion de Crespigny |
| author_sort | A. Krelle |
| collection | DOAJ |
| description | Thrombotic microangiopathy (TMA) reflects a syndrome of endothelial injury characterised by microangiopathic haemolytic anaemia (nonimmune), thrombocytopenia, and often end-organ dysfunction. TMA disorders are well-recognised in kidney transplant recipients, often due to an underlying genetic predisposition related to complement dysregulation, or de novo due to infection, immunosuppression toxicity, or antibody-mediated rejection. In pregnancy, TMA disorders are most commonly due to severe pre-eclampsia or HELLP, but may also be due to thrombotic thrombocytopenic purpura (TTP) or complement-mediated (atypical) haemolytic uremic syndrome (aHUS). Complement dysregulation is being recognised as playing a role in the development of preeclampsia and HELLP syndrome in addition to aHUS. Due to overlapping clinical and laboratory features, diagnosis can be difficult and delays in treatment can be life-threatening for both mother and fetus. This report describes a 32 year-old female who had two successive wanted pregnancies. The first pregnancy was terminated at 22 weeks gestation due to presumed severe preeclampsia and fetal growth restriction in the context of known chronic kidney failure due to reflux nephropathy. A living-related kidney transplant was performed to improve the chances of pregnancy resulting in a live birth. A subsequent pregnancy was complicated by progressive kidney impairment and hypertension at 22 weeks gestation. Kidney biopsy showed TMA, but the etiology was unclear. This report highlights the diagnostic dilemma of TMA in a pregnant kidney transplant recipient and a role for the anti-C5 terminal complement blockade monoclonal antibody eculizumab, in pregnancy-associated TMA, especially at a peri-viable gestation. |
| format | Article |
| id | doaj-art-d6321e48464f4587ace1ba70e30e8577 |
| institution | Kabale University |
| issn | 2090-665X |
| language | English |
| publishDate | 2024-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Nephrology |
| spelling | doaj-art-d6321e48464f4587ace1ba70e30e85772025-02-03T05:55:20ZengWileyCase Reports in Nephrology2090-665X2024-01-01202410.1155/2024/9218637The Challenges of Distinguishing Different Causes of TMA in a Pregnant Kidney Transplant RecipientA. Krelle0S. Price1M. M. Law2S. Kranz3P. Shamdasani4S. Kane5J. Unterscheider6P. Champion de Crespigny7Department of Obstetric MedicineDepartment of Obstetric MedicineDepartment of Obstetric MedicineDepartment of Anatomical PathologyDepartment of Obstetric MedicineDepartment of Maternal Fetal MedicineDepartment of Maternal Fetal MedicineDepartment of Obstetric MedicineThrombotic microangiopathy (TMA) reflects a syndrome of endothelial injury characterised by microangiopathic haemolytic anaemia (nonimmune), thrombocytopenia, and often end-organ dysfunction. TMA disorders are well-recognised in kidney transplant recipients, often due to an underlying genetic predisposition related to complement dysregulation, or de novo due to infection, immunosuppression toxicity, or antibody-mediated rejection. In pregnancy, TMA disorders are most commonly due to severe pre-eclampsia or HELLP, but may also be due to thrombotic thrombocytopenic purpura (TTP) or complement-mediated (atypical) haemolytic uremic syndrome (aHUS). Complement dysregulation is being recognised as playing a role in the development of preeclampsia and HELLP syndrome in addition to aHUS. Due to overlapping clinical and laboratory features, diagnosis can be difficult and delays in treatment can be life-threatening for both mother and fetus. This report describes a 32 year-old female who had two successive wanted pregnancies. The first pregnancy was terminated at 22 weeks gestation due to presumed severe preeclampsia and fetal growth restriction in the context of known chronic kidney failure due to reflux nephropathy. A living-related kidney transplant was performed to improve the chances of pregnancy resulting in a live birth. A subsequent pregnancy was complicated by progressive kidney impairment and hypertension at 22 weeks gestation. Kidney biopsy showed TMA, but the etiology was unclear. This report highlights the diagnostic dilemma of TMA in a pregnant kidney transplant recipient and a role for the anti-C5 terminal complement blockade monoclonal antibody eculizumab, in pregnancy-associated TMA, especially at a peri-viable gestation.http://dx.doi.org/10.1155/2024/9218637 |
| spellingShingle | A. Krelle S. Price M. M. Law S. Kranz P. Shamdasani S. Kane J. Unterscheider P. Champion de Crespigny The Challenges of Distinguishing Different Causes of TMA in a Pregnant Kidney Transplant Recipient Case Reports in Nephrology |
| title | The Challenges of Distinguishing Different Causes of TMA in a Pregnant Kidney Transplant Recipient |
| title_full | The Challenges of Distinguishing Different Causes of TMA in a Pregnant Kidney Transplant Recipient |
| title_fullStr | The Challenges of Distinguishing Different Causes of TMA in a Pregnant Kidney Transplant Recipient |
| title_full_unstemmed | The Challenges of Distinguishing Different Causes of TMA in a Pregnant Kidney Transplant Recipient |
| title_short | The Challenges of Distinguishing Different Causes of TMA in a Pregnant Kidney Transplant Recipient |
| title_sort | challenges of distinguishing different causes of tma in a pregnant kidney transplant recipient |
| url | http://dx.doi.org/10.1155/2024/9218637 |
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