Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab
TAFRO syndrome, a rare subtype of idiopathic multicentric Castleman disease, manifests as thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Thrombotic microangiopathy, including renal dysfunction, is frequently associated with this syndrome. TAFRO syndrome can be life threaten...
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| Format: | Article |
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MDPI AG
2021-09-01
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| Series: | Medicina |
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| Online Access: | https://www.mdpi.com/1648-9144/57/9/971 |
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| author | Yuina Akagi Takashi Kato Yusuke Yamashita Hiroki Hosoi Shogo Murata Shuto Yamamoto Kenji Warigaya Taisei Nakao Shinichi Murata Takashi Sonoki Shinobu Tamura |
| author_facet | Yuina Akagi Takashi Kato Yusuke Yamashita Hiroki Hosoi Shogo Murata Shuto Yamamoto Kenji Warigaya Taisei Nakao Shinichi Murata Takashi Sonoki Shinobu Tamura |
| author_sort | Yuina Akagi |
| collection | DOAJ |
| description | TAFRO syndrome, a rare subtype of idiopathic multicentric Castleman disease, manifests as thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Thrombotic microangiopathy, including renal dysfunction, is frequently associated with this syndrome. TAFRO syndrome can be life threatening and show rapid progression, and the diagnosis and management of this disorder remain challenging. A 48-year-old woman was diagnosed with TAFRO syndrome complicated by thrombotic microangiopathy based on the clinical and histopathological findings. After receiving high-dose steroids, her thrombocytopenia and anasarca did not improve. The patient subsequently received a combination of cyclosporine A and rituximab as second-line therapy, which resulted in a significant gradual improvement in the clinical symptoms. Meanwhile, her platelet count increased to more than 40 × 10<sup>9</sup>/L; however, she developed intracranial hemorrhage. Following surgical evacuation, the patient recovered with an achievement of sustained remission. Based on these findings, attention should be paid to life-threatening bleeding associated with local thrombotic microangiopathy even when intensive treatment is administered for TAFRO syndrome. |
| first_indexed | 2024-03-10T07:28:31Z |
| format | Article |
| id | doaj.art-efdcefee95934613911d0451586cfb27 |
| institution | Directory Open Access Journal |
| issn | 1010-660X 1648-9144 |
| language | English |
| last_indexed | 2024-03-10T07:28:31Z |
| publishDate | 2021-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Medicina |
| spelling | doaj.art-efdcefee95934613911d0451586cfb272023-11-22T14:09:06ZengMDPI AGMedicina1010-660X1648-91442021-09-0157997110.3390/medicina57090971Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and RituximabYuina Akagi0Takashi Kato1Yusuke Yamashita2Hiroki Hosoi3Shogo Murata4Shuto Yamamoto5Kenji Warigaya6Taisei Nakao7Shinichi Murata8Takashi Sonoki9Shinobu Tamura10Department of Hematology/Oncology, Wakayama Medical University, Wakayama 6418509, JapanDepartment of Hematology/Oncology, Wakayama Medical University, Wakayama 6418509, JapanDepartment of Hematology/Oncology, Wakayama Medical University, Wakayama 6418509, JapanDepartment of Hematology/Oncology, Wakayama Medical University, Wakayama 6418509, JapanDepartment of Hematology/Oncology, Wakayama Medical University, Wakayama 6418509, JapanDepartment of Nephrology, Wakayama Medical University, Wakayama 6418509, JapanDepartment of Diagnostic Pathology, Wakayama Medical University, Wakayama 6418509, JapanDepartment of Internal Medicine, Naga Municipal Hospital, Wakayama 6496414, JapanDepartment of Diagnostic Pathology, Wakayama Medical University, Wakayama 6418509, JapanDepartment of Hematology/Oncology, Wakayama Medical University, Wakayama 6418509, JapanDepartment of Hematology/Oncology, Wakayama Medical University, Wakayama 6418509, JapanTAFRO syndrome, a rare subtype of idiopathic multicentric Castleman disease, manifests as thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Thrombotic microangiopathy, including renal dysfunction, is frequently associated with this syndrome. TAFRO syndrome can be life threatening and show rapid progression, and the diagnosis and management of this disorder remain challenging. A 48-year-old woman was diagnosed with TAFRO syndrome complicated by thrombotic microangiopathy based on the clinical and histopathological findings. After receiving high-dose steroids, her thrombocytopenia and anasarca did not improve. The patient subsequently received a combination of cyclosporine A and rituximab as second-line therapy, which resulted in a significant gradual improvement in the clinical symptoms. Meanwhile, her platelet count increased to more than 40 × 10<sup>9</sup>/L; however, she developed intracranial hemorrhage. Following surgical evacuation, the patient recovered with an achievement of sustained remission. Based on these findings, attention should be paid to life-threatening bleeding associated with local thrombotic microangiopathy even when intensive treatment is administered for TAFRO syndrome.https://www.mdpi.com/1648-9144/57/9/971TAFRO syndromethrombotic microangiopathycyclosporine Arituximabintracranial hemorrhage |
| spellingShingle | Yuina Akagi Takashi Kato Yusuke Yamashita Hiroki Hosoi Shogo Murata Shuto Yamamoto Kenji Warigaya Taisei Nakao Shinichi Murata Takashi Sonoki Shinobu Tamura Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab Medicina TAFRO syndrome thrombotic microangiopathy cyclosporine A rituximab intracranial hemorrhage |
| title | Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab |
| title_full | Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab |
| title_fullStr | Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab |
| title_full_unstemmed | Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab |
| title_short | Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab |
| title_sort | intracranial hemorrhage in a patient with tafro syndrome treated with cyclosporine a and rituximab |
| topic | TAFRO syndrome thrombotic microangiopathy cyclosporine A rituximab intracranial hemorrhage |
| url | https://www.mdpi.com/1648-9144/57/9/971 |
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