TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report
A 57-year-old man who received a kidney transplant 4 years previously owing to unknown underlying disease presented with thrombocytopenia and fever. Hepatosplenomegaly and lymphadenopathy were observed, and development of prominent anasarca and worsening of renal function yielded the diagnosis of TA...
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Frontiers Media S.A.
2021-10-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2021.747678/full |
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| author | Marie Nagai Takahiro Uchida Muneharu Yamada Shuuhei Komatsu Kohei Ota Mitsuya Mukae Hitoshi Iwamoto Hiroshi Hirano Miho Karube Shinya Kaname Takashi Oda |
| author_facet | Marie Nagai Takahiro Uchida Muneharu Yamada Shuuhei Komatsu Kohei Ota Mitsuya Mukae Hitoshi Iwamoto Hiroshi Hirano Miho Karube Shinya Kaname Takashi Oda |
| author_sort | Marie Nagai |
| collection | DOAJ |
| description | A 57-year-old man who received a kidney transplant 4 years previously owing to unknown underlying disease presented with thrombocytopenia and fever. Hepatosplenomegaly and lymphadenopathy were observed, and development of prominent anasarca and worsening of renal function yielded the diagnosis of TAFRO syndrome. He was treated with high-dose steroids and plasmapheresis, and a thrombopoietin receptor agonist was administered for refractory thrombocytopenia. However, his general condition worsened, and he died on day 92. Histopathological analysis of a kidney autopsy specimen showed thrombotic microangiopathy characterized by glomerular endothelial swelling, mesangiolysis, and double contours of the glomerular capillary walls. His bone marrow showed megakaryocytic hyperplasia with mild reticulin fibrosis. Interestingly, these clinical and pathological features were remarkably similar to those the patient demonstrated before the kidney transplant, suggesting the recurrence of TAFRO syndrome. TAFRO syndrome is a rare systemic disorder whose concept has recently been established, but information on its long-term outcome is scarce. To our knowledge, this is the first case of TAFRO syndrome developing in a kidney transplant recipient, which suggests that disease recurrence occurs many years after the kidney transplant. |
| first_indexed | 2024-12-13T19:53:35Z |
| format | Article |
| id | doaj.art-14473e3c3e5f44f88129f39dcbcf83e0 |
| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-12-13T19:53:35Z |
| publishDate | 2021-10-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Medicine |
| spelling | doaj.art-14473e3c3e5f44f88129f39dcbcf83e02022-12-21T23:33:22ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2021-10-01810.3389/fmed.2021.747678747678TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case ReportMarie Nagai0Takahiro Uchida1Muneharu Yamada2Shuuhei Komatsu3Kohei Ota4Mitsuya Mukae5Hitoshi Iwamoto6Hiroshi Hirano7Miho Karube8Shinya Kaname9Takashi Oda10Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, JapanDepartment of Kidney Transplantation Surgery, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, JapanDepartmet of Diagnostic Pathology, Tokyo Medical University Hachioji Medical Center, Hachioji, JapanDepartment of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo, JapanDepartment of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, JapanA 57-year-old man who received a kidney transplant 4 years previously owing to unknown underlying disease presented with thrombocytopenia and fever. Hepatosplenomegaly and lymphadenopathy were observed, and development of prominent anasarca and worsening of renal function yielded the diagnosis of TAFRO syndrome. He was treated with high-dose steroids and plasmapheresis, and a thrombopoietin receptor agonist was administered for refractory thrombocytopenia. However, his general condition worsened, and he died on day 92. Histopathological analysis of a kidney autopsy specimen showed thrombotic microangiopathy characterized by glomerular endothelial swelling, mesangiolysis, and double contours of the glomerular capillary walls. His bone marrow showed megakaryocytic hyperplasia with mild reticulin fibrosis. Interestingly, these clinical and pathological features were remarkably similar to those the patient demonstrated before the kidney transplant, suggesting the recurrence of TAFRO syndrome. TAFRO syndrome is a rare systemic disorder whose concept has recently been established, but information on its long-term outcome is scarce. To our knowledge, this is the first case of TAFRO syndrome developing in a kidney transplant recipient, which suggests that disease recurrence occurs many years after the kidney transplant.https://www.frontiersin.org/articles/10.3389/fmed.2021.747678/fullautopsykidney transplantmembranoproliferative glomerulonephritisTAFRO syndromethrombotic microangiopathy |
| spellingShingle | Marie Nagai Takahiro Uchida Muneharu Yamada Shuuhei Komatsu Kohei Ota Mitsuya Mukae Hitoshi Iwamoto Hiroshi Hirano Miho Karube Shinya Kaname Takashi Oda TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report Frontiers in Medicine autopsy kidney transplant membranoproliferative glomerulonephritis TAFRO syndrome thrombotic microangiopathy |
| title | TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report |
| title_full | TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report |
| title_fullStr | TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report |
| title_full_unstemmed | TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report |
| title_short | TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report |
| title_sort | tafro syndrome in a kidney transplant recipient that was diagnosed on autopsy a case report |
| topic | autopsy kidney transplant membranoproliferative glomerulonephritis TAFRO syndrome thrombotic microangiopathy |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2021.747678/full |
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