TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 Therapy
TAFRO syndrome is defined by the presence of thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis/renal dysfunction (R), and organomegaly (O) and can be seen with idiopathic multicentric Castleman disease (iMCD) or as an isolated process without iMCD. Although the diagnosis of iMCD in p...
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| Format: | Article |
| Language: | English |
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Karger Publishers
2021-09-01
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| Series: | Case Reports in Oncology |
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| Online Access: | https://www.karger.com/Article/FullText/518079 |
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| author | Corinne Williams Alexis Phillips Vikram Aggarwal Liron Barnea Slonim David C. Fajgenbaum Reem Karmali |
| author_facet | Corinne Williams Alexis Phillips Vikram Aggarwal Liron Barnea Slonim David C. Fajgenbaum Reem Karmali |
| author_sort | Corinne Williams |
| collection | DOAJ |
| description | TAFRO syndrome is defined by the presence of thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis/renal dysfunction (R), and organomegaly (O) and can be seen with idiopathic multicentric Castleman disease (iMCD) or as an isolated process without iMCD. Although the diagnosis of iMCD in patients with TAFRO can be challenging to make, iMCD should remain high on the differential diagnosis. Similar to iMCD, the pathophysiology of TAFRO is not well understood but is thought to be related to hypercytokinemia, with interleukin (IL)-6 playing a pivotal role. Anti-IL-6 monoclonal antibody therapy is an effective treatment modality for iMCD, but to date, there is no clear guidance on treatment of TAFRO in the absence of definitive diagnosis of iMCD, leading to suboptimal management and high morbidity. We report a case of TAFRO syndrome and demonstrate benefit with the empiric use of anti-IL-6 antibody therapy in the context of delayed diagnosis of iMCD. |
| first_indexed | 2024-12-16T17:52:51Z |
| format | Article |
| id | doaj.art-765da5424ebd4c369571f06960ae50a5 |
| institution | Directory Open Access Journal |
| issn | 1662-6575 |
| language | English |
| last_indexed | 2024-12-16T17:52:51Z |
| publishDate | 2021-09-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Oncology |
| spelling | doaj.art-765da5424ebd4c369571f06960ae50a52022-12-21T22:22:15ZengKarger PublishersCase Reports in Oncology1662-65752021-09-011431359136510.1159/000518079518079TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 TherapyCorinne Williams0Alexis Phillips1Vikram Aggarwal2Liron Barnea Slonim3David C. Fajgenbaum4Reem Karmali5Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, IL, USAUniversity of Pennsylvania, Philadelphia, PA, USADivision of Nephrology and Hypertension, Northwestern University Feinberg School of Medicine, Chicago, IL, USADivision of Pathology, Northwestern University, Chicago, IL, USATranslational Medicine & Human Genetics, University of Pennsylvania, Philadelphia, PA, USARobert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, IL, USATAFRO syndrome is defined by the presence of thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis/renal dysfunction (R), and organomegaly (O) and can be seen with idiopathic multicentric Castleman disease (iMCD) or as an isolated process without iMCD. Although the diagnosis of iMCD in patients with TAFRO can be challenging to make, iMCD should remain high on the differential diagnosis. Similar to iMCD, the pathophysiology of TAFRO is not well understood but is thought to be related to hypercytokinemia, with interleukin (IL)-6 playing a pivotal role. Anti-IL-6 monoclonal antibody therapy is an effective treatment modality for iMCD, but to date, there is no clear guidance on treatment of TAFRO in the absence of definitive diagnosis of iMCD, leading to suboptimal management and high morbidity. We report a case of TAFRO syndrome and demonstrate benefit with the empiric use of anti-IL-6 antibody therapy in the context of delayed diagnosis of iMCD.https://www.karger.com/Article/FullText/518079idiopathic multicentric castleman diseasetafrointerleukin-6siltuximabtreatment |
| spellingShingle | Corinne Williams Alexis Phillips Vikram Aggarwal Liron Barnea Slonim David C. Fajgenbaum Reem Karmali TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 Therapy Case Reports in Oncology idiopathic multicentric castleman disease tafro interleukin-6 siltuximab treatment |
| title | TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 Therapy |
| title_full | TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 Therapy |
| title_fullStr | TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 Therapy |
| title_full_unstemmed | TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 Therapy |
| title_short | TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 Therapy |
| title_sort | tafro syndrome and elusive diagnosis of idiopathic multicentric castleman disease treated with empiric anti interleukin 6 therapy |
| topic | idiopathic multicentric castleman disease tafro interleukin-6 siltuximab treatment |
| url | https://www.karger.com/Article/FullText/518079 |
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