A Case of TAFRO Syndrome Successfully Treated with Intravenous Cyclophosphamide Therapy

Suzuki, Kazumasa; Nakamura, Kazuko; Kasuya, Tadamichi; Yamasaki, Kenya; Tokinaga, Kotaro; Tashiro, Jun; Kimura, Akira; Noro, Masahiro; Akikusa, Bunshiro; Kojima, Masaru; Umibe, Takeshi

doi:10.2169/naika.105.2417

Cited by 3 publications

(1 citation statement)

References 8 publications

(1 reference statement)

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“…There is no standard therapy for TAFRO syndrome, but in Japan, glucocorticoid therapy is usually administered (4). In cases refractory to glucocorticoid therapy, other immunosuppressants, such as cyclosporin, rituximab, cyclophosphamide (CPA), and tocilizumab, have been prescribed (4)(5)(6)(7).…”

Section: Introductionmentioning

confidence: 99%

TAFRO Syndrome Presenting with Retroperitoneal Panniculitis-like Computed Tomography Findings at Disease Onset

Yanagiya

Suzuki²,

Nakamura

et al. 2020

Intern. Med.

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TAFRO syndrome is rare, and its pathophysiology remains unclear. We herein report the case of a 66-yearold man who presented at our emergency department with epigastric pain. Contrast-enhanced computed tomography (CT) showed high-density retroperitoneal panniculus with contrast enhancement. He was treated initially with a protease inhibitor and hydration, to little effect. Anasarca, thrombocytopenia, and renal dysfunction developed gradually, and TAFRO syndrome was diagnosed. He was treated successfully with prednisolone and cyclophosphamide, and subsequent CT findings showed improvement. Abnormal CT findings of the retroperitoneum may indicate the early stages of TAFRO syndrome before the presentation of typical ascites.

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Section: Introductionmentioning

confidence: 99%

TAFRO Syndrome Presenting with Retroperitoneal Panniculitis-like Computed Tomography Findings at Disease Onset

Yanagiya

Suzuki²,

Nakamura

et al. 2020

Intern. Med.

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Pathophysiology, Treatment, and Prognosis of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome: A Review

Kakutani,

Kamada,

Tamai

2024

CIMB

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TAFRO syndrome, first reported in 2010, is a systemic inflammatory disease with a rapid onset and potentially fatal course if not treated promptly and appropriately. The name is derived from the initial letters describing the characteristic symptoms of thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It is sometimes considered a special subtype of idiopathic multicentric Castleman disease (iMCD) because lymph node biopsies often reveal the pathology findings seen in iMCD. However, its clinical manifestations and prognoses are not well documented. Since the clinical manifestations and prognoses of TAFRO syndrome differ significantly from those of iMCD, it is recognized as an independent disease concept and considered to partially overlap with the pathology of MCD. The pathogenesis of TAFRO syndrome remains largely unknown. Due to the lack of appropriate treatment, it often presents with multiple organ dysfunction and fatality. In this review, we summarized new findings on the pathogenesis of TAFRO syndrome and discussed current effective therapies and future treatment strategies.

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International definition of iMCD-TAFRO: future perspectives

Nishimura

Sato

2022

JCEH

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Since thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly (TAFRO) syndrome was first proposed in 2010, there has been considerable progress in this area, particularly regarding its association with idiopathic multicentric Castleman disease (iMCD). TAFRO syndrome is a heterogeneous category with a constellation of symptoms that can develop in the setting of infection, rheumatologic disorder, malignancy, and iMCD. Now, iMCD with TAFRO symptoms is subtyped as iMCD-TAFRO. However, confusion between TAFRO syndrome and iMCD-TAFRO remains. In this article, we discuss the current understanding and future research agenda of TAFRO syndrome and iMCD-TAFRO from the perspective of its new validated international definition.

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A Case of TAFRO Syndrome Successfully Treated with Intravenous Cyclophosphamide Therapy

Cited by 3 publications

References 8 publications

TAFRO Syndrome Presenting with Retroperitoneal Panniculitis-like Computed Tomography Findings at Disease Onset

TAFRO Syndrome Presenting with Retroperitoneal Panniculitis-like Computed Tomography Findings at Disease Onset

Pathophysiology, Treatment, and Prognosis of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome: A Review

International definition of iMCD-TAFRO: future perspectives

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