A case report of TAFRO syndrome successfully treated by immunosuppressive therapies with plasma exchange

Meguri, Yusuke; Asada, Noboru; Nakasako, Yukio; Kondo, Eisei; Kambara, Yui; Yamamoto, Akira; Masunari, Taro; Sezaki, Nobuo; Ikeda, Genyo; Toji, Tomohiro; Yoshino, Tadashi; Kiguchi, Toru

doi:10.1007/s00277-018-3456-9

Cited by 12 publications

(10 citation statements)

References 10 publications

(12 reference statements)

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“…In addition to the core TAFRO signs and symptoms, elevation of ALP is also a common abnormality; however, liver damage and hyperbilirubinemia have not been mentioned in review papers ( 2 , 14 , 20 ). There are two reports of cases with hyperbilirubinemia ( 12 , 21 ), but a liver biopsy was not conducted in either. Seven reports described cases with liver damage without hyperbilirubinemia and mentioned liver histology in patients with TAFRO syndrome ( 5 , 6 , 22 - 24 ); five studies reported no specific histological findings, one reported interface hepatitis with infiltration of inflammatory cells ( 6 ), and the remaining study reported lobular hepatitis with no steatosis ( 5 ).…”

Section: Discussionmentioning

confidence: 99%

“…Immunosuppressive and anti-inflammatory therapies are standard for TAFRO syndrome. CS suppress the inflammatory processes regulated by cytokines and chemokines, including IL-6 and VEGF, which play crucial roles in TAFRO syndrome, and have been used as the first treatment in most reported cases ( 2 , 12 ). If steroid monotherapy does not demonstrate sufficient efficacy, additional treatment is required.…”

Section: Discussionmentioning

confidence: 99%

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Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia

et al. 2021

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TAFRO syndrome is a systemic inflammatory, lymphoproliferative disorder, but the pathophysiology of the disease is unknown. It is typically characterized by thrombocytopenia, anasarca, a fever, reticulin fibrosis, renal dysfunction, and organomegaly. However, other manifestations have been also reported. We encountered a 43-year-old man with TAFRO syndrome who showed mediastinal panniculitis, liver damage, and adrenal lesions in addition to the core signs. He achieved complete remission with combination therapy of corticosteroids, tocilizumab, and cyclosporin, and remission was maintained even after drug discontinuation at 15 months. Atypical manifestations and complete remission of TAFRO syndrome were remarkable features of our case.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia

et al. 2021

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“…Consistent with this idea, successful treatment using rituximab and plasma exchange has been reported in a previous case of TAFRO syndrome. 23 …”

Section: Discussionmentioning

confidence: 99%

“…Consistent with this idea, successful treatment using rituximab and plasma exchange has been reported in a previous case of TAFRO syndrome. 23 The factors that determine the prognosis of TAFRO syndrome are unknown. Disease severity scores for TAFRO syndrome are based on the combination of thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal insufficiency, organomegaly, and other clinically identifiable findings.…”

Section: Discussionmentioning

confidence: 99%

TAFRO Syndrome With Kidney Involvement: A Case Series of Patients With Kidney Biopsies

et al. 2021

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TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal insufficiency, and organomegaly) syndrome is a systemic inflammatory disease sharing some features with Castleman disease and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome in relation to abnormal secretions of interleukin 6 and vascular endothelial growth factor. The kidney is a main target organ of TAFRO syndrome but the kidney histopathology associated with TAFRO syndrome is yet to be completely defined. We report 3 TAFRO syndrome cases with different clinical courses in which kidney biopsies were performed. In all 3 cases, kidney biopsies showed similar glomerular lesions of diffuse global swelling of the endothelium and expansion of subendothelial spaces, consistent with severe glomerular endothelial injury. Case 3 showed an additional finding of focal tubulointerstitial injury characterized by marked plasma cell infiltration, which was absent in the other 2 cases. Clinical symptoms in cases 1 and 2, which had lower disease severity scores of TAFRO syndrome, were effectively treated with the administration of corticosteroids or a combination of corticosteroids and cyclosporine A. Case 3, with a higher disease severity score, had an aggressive clinical course that was refractory to corticosteroids and tocilizumab; the patient ultimately died of multiple organ failure. In all 3 cases, kidney biopsy provided indications for the diagnosis process and clinical management of TAFRO syndrome.

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“…Due to a scarcity of cases and a lack of understanding of the disease mechanisms, there is no standard treatment for TAFRO syndrome. The main therapeutic options include corticosteroids, immunosuppressive therapy, rituximab or rituximab-based therapy, anti-IL-6 therapies (tocilizumab and siltuximab), thalidomide combined with cyclophosphamide and prednisone, and plasma exchange [3,5,6,9,10,[19][20][21][22]. The Japanese TAFRO Research Group recommends high-dose steroids, tocilizumab, and cyclosporine A for TAFRO patients [7].…”

Section: Case Presentationmentioning

confidence: 99%

Acute Kidney Injury Caused by TAFRO Syndrome in a Chinese Patient: Efficacy of Long-Term Corticosteroids Combined with Bortezomib and Cyclophosphamide

Xia

Zhang

Zou

et al. 2020

Kidney Blood Press Res

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Introduction: Thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a newly recognized and rare clinical subtype of Castleman disease. Renal involvement in TAFRO syndrome usually presents with mild proteinuria, microscopic hematuria, and acute renal injury requiring temporary renal replacement. There is no standard therapy available and treatment failures are common, leading to a poor prognosis. We report a case of acute renal failure caused by TAFRO syndrome, successfully managed by long-term corticosteroids combined with bortezomib and cyclophosphamide. Case Presentation: The patient was a 52-year-old female who presented with fever, anasarca, oliguria, and abdominal distension at first. She progressed rapidly to anuric renal failure requiring hemodialysis. She also demonstrated thrombocytopenia, anemia, coagulopathy, and a hyperinflammatory status. Her CT scan showed severe polyserositis, splenomegaly, and lymphadenopathy. Her serum vascular epithelial growth factor level was significantly elevated. Axillary lymph node biopsy showed hyaline-vascular type Castleman disease, supporting the diagnosis of TAFRO

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A case report of TAFRO syndrome successfully treated by immunosuppressive therapies with plasma exchange

Cited by 12 publications

References 10 publications

Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia

Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia

TAFRO Syndrome With Kidney Involvement: A Case Series of Patients With Kidney Biopsies

Acute Kidney Injury Caused by TAFRO Syndrome in a Chinese Patient: Efficacy of Long-Term Corticosteroids Combined with Bortezomib and Cyclophosphamide

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