To and TAFRO – a cryptic cause of acute renal failure: a case report
Abstract Background TAFRO syndrome is a rare clinical subtype of idiopathic multicentric Castlemans disease characterised by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. Renal involvement is common, sometimes requiring temporary renal replacement therapy. Due to th...
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| Format: | Article |
| Language: | English |
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BMC
2022-01-01
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| Series: | BMC Nephrology |
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| Online Access: | https://doi.org/10.1186/s12882-022-02660-7 |
| _version_ | 1818940662778691584 |
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| author | N. Shah T. Davidson C. Cheung K. Keung |
| author_facet | N. Shah T. Davidson C. Cheung K. Keung |
| author_sort | N. Shah |
| collection | DOAJ |
| description | Abstract Background TAFRO syndrome is a rare clinical subtype of idiopathic multicentric Castlemans disease characterised by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. Renal involvement is common, sometimes requiring temporary renal replacement therapy. Due to the associated thrombocytopenia, renal biopsies are rarely performed limiting descriptions of the renal histopathology in this condition. This case describes a patient with TAFRO syndrome and the associated renal histology. Case presentation A 49-year-old Caucasian man presented to a tertiary hospital in Sydney with a six- week history of malaise, non-bloody diarrhoea, progressive shortness of breath, and drenching night sweats. A progressive bicytopenia and renal function decline necessitating temporary dialysis prompted a bone marrow aspirate and trephine, as well as a renal biopsy respectively. This noted a hypercellular bone marrow with increased granulopoiesis, reduced erythropoiesis, and fibrosis, with renal histology suggesting a thrombotic microangiopathic-like glomerulopathy. Alternate conditions were excluded, and a diagnosis of TAFRO syndrome was made. Glucocorticoids and rituximab were initiated with rapid renal recovery, and normalisation of his haematologic parameters achieved at six months. Conclusion This case describes an atypical thrombotic microangiopathy as the predominant histologic renal lesion in a patient with TAFRO syndrome. This was responsive to immunosuppression with glucocorticoids and rituximab, highlighting the importance of early recognition of this rarely described condition. |
| first_indexed | 2024-12-20T06:43:13Z |
| format | Article |
| id | doaj.art-6d7552ba5ed448388d66a33e6939e730 |
| institution | Directory Open Access Journal |
| issn | 1471-2369 |
| language | English |
| last_indexed | 2024-12-20T06:43:13Z |
| publishDate | 2022-01-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Nephrology |
| spelling | doaj.art-6d7552ba5ed448388d66a33e6939e7302022-12-21T19:49:48ZengBMCBMC Nephrology1471-23692022-01-012311510.1186/s12882-022-02660-7To and TAFRO – a cryptic cause of acute renal failure: a case reportN. Shah0T. Davidson1C. Cheung2K. Keung3Department of Nephrology, Prince of Wales HospitalNew South Wales Health Pathology East, Prince of Wales HospitalDepartment of Haematology, Prince of Wales HospitalDepartment of Nephrology, Prince of Wales HospitalAbstract Background TAFRO syndrome is a rare clinical subtype of idiopathic multicentric Castlemans disease characterised by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. Renal involvement is common, sometimes requiring temporary renal replacement therapy. Due to the associated thrombocytopenia, renal biopsies are rarely performed limiting descriptions of the renal histopathology in this condition. This case describes a patient with TAFRO syndrome and the associated renal histology. Case presentation A 49-year-old Caucasian man presented to a tertiary hospital in Sydney with a six- week history of malaise, non-bloody diarrhoea, progressive shortness of breath, and drenching night sweats. A progressive bicytopenia and renal function decline necessitating temporary dialysis prompted a bone marrow aspirate and trephine, as well as a renal biopsy respectively. This noted a hypercellular bone marrow with increased granulopoiesis, reduced erythropoiesis, and fibrosis, with renal histology suggesting a thrombotic microangiopathic-like glomerulopathy. Alternate conditions were excluded, and a diagnosis of TAFRO syndrome was made. Glucocorticoids and rituximab were initiated with rapid renal recovery, and normalisation of his haematologic parameters achieved at six months. Conclusion This case describes an atypical thrombotic microangiopathy as the predominant histologic renal lesion in a patient with TAFRO syndrome. This was responsive to immunosuppression with glucocorticoids and rituximab, highlighting the importance of early recognition of this rarely described condition.https://doi.org/10.1186/s12882-022-02660-7Acute renal failureKidney biopsyCase report |
| spellingShingle | N. Shah T. Davidson C. Cheung K. Keung To and TAFRO – a cryptic cause of acute renal failure: a case report BMC Nephrology Acute renal failure Kidney biopsy Case report |
| title | To and TAFRO – a cryptic cause of acute renal failure: a case report |
| title_full | To and TAFRO – a cryptic cause of acute renal failure: a case report |
| title_fullStr | To and TAFRO – a cryptic cause of acute renal failure: a case report |
| title_full_unstemmed | To and TAFRO – a cryptic cause of acute renal failure: a case report |
| title_short | To and TAFRO – a cryptic cause of acute renal failure: a case report |
| title_sort | to and tafro a cryptic cause of acute renal failure a case report |
| topic | Acute renal failure Kidney biopsy Case report |
| url | https://doi.org/10.1186/s12882-022-02660-7 |
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