A Case of Repeated TAFRO Syndrome-Like Symptoms and Retroperitoneal Hemorrhage in a Patient With Sjögren Syndrome

Ohta, Takanori; Oda, Naoki; Saito, Keiko; Tamiya, Sadafumi; Ueno, Tomoko

doi:10.7759/cureus.12175

Cureus

2020

DOI: 10.7759/cureus.12175

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A Case of Repeated TAFRO Syndrome-Like Symptoms and Retroperitoneal Hemorrhage in a Patient With Sjögren Syndrome

Takanori Ohta¹,

Naoki Oda²,

Keiko Saito³

et al.

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2022

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Cited by 2 publications

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“…23 To date, there have been several reports describing that autoimmune diseases, such as SjS and systemic lupus erythematosus (SLE), present as TAFRO syndrome without iMCD. [24][25][26][27][28][29][30][31][32] At the same time, these reports raise a concern that there may be cases of overlooked autoimmune diseases misdiagnosed as iMCD. Considering the difference in treatment strategies, detecting atypical autoimmune diseases among iMCD is essential.…”

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confidence: 99%

Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?

Nishimura

Nishikori

Sawada

et al. 2022

JCEH

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Idiopathic multicentric Castleman disease (iMCD) is a rare, clinicopathologically heterogeneous disorder with systemic inflammation and/or multi-organ dysfunction related to a high pro-inflammatory cytokine status. 1 Since Fajgenbaum et al. proposed the international consensus diagnostic criteria for iMCD, there has been less confusion regarding diagnostic and treatment approaches for the disorder. 2 However, iMCD has considerable heterogeneity. 3 Pathologically, iMCD is divided into plasma cell (PC) type, hypervascular (HV) type, and a mixed variant, although the histological definition of the mixed variant is unclear. The pathology of PC-type iMCD (PC-iMCD) is characterized by atrophic to hyperplastic germinal centers, interfollicular plasmacytosis, and mild to severe vascularity. Clinically, patients with iMCD are further classified into iMCD not otherwise specified (iMCD-NOS) or thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (iMCD-TAFRO). TAFRO syndrome is a heterogeneous entity with a constellation of the aforementioned symptoms, including infectious diseases,

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mentioning

confidence: 99%

Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?

Nishimura

Nishikori

Sawada

et al. 2022

JCEH

View full text Add to dashboard Cite

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TAFRO syndrome: A disease that known is half cured

Chen

Feng

Zhang

et al. 2022

Hematological Oncology

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Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly (TAFRO) syndrome is rare in clinical practice. It is a systemic inflammatory disease caused by a cytokine storm. Its clinical manifestations include thrombocytopenia, systemic edema, fever, bone marrow fibrosis, renal insufficiency, and organ enlargement. The high mortality rate of TAFRO syndrome is due to the difficulty of acquiring biopsy samples for diagnosis and the rapid disease progression. This disease is poorly understood by clinicians. Early detection, accurate diagnosis, and timely treatment play key roles in prolonging the survival of the patients. This review summarizes the latest progress in the pathogenesis, diagnostic criteria, and treatment regimens of TAFRO syndrome, aiming to help clinicians better understand TAFRO syndrome and improve its diagnosis and treatment.

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A Case of Repeated TAFRO Syndrome-Like Symptoms and Retroperitoneal Hemorrhage in a Patient With Sjögren Syndrome

Cited by 2 publications

References 20 publications

Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?

Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?

TAFRO syndrome: A disease that known is half cured

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