TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly) 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. This article is protected by copyright. All rights reserved.

TAFRO Syndrome: A Disease that Known is Half Cured.

Resume Introduction Le syndrome TAFRO est un syndrome inflammatoire systemique proche de la maladie de Castleman, associant, anasarque, fievre, insuffisance renale et/ou fibrose reticulinique et organomegalie. Nous rapportons la premiere observation de vascularite necrosante compliquant un syndrome TAFRO. Observation Une femme de 69 ans presentait amaigrissement, fievre, anasarque, splenomegalie, adenomegalies, oligoanurie et livedo necrotique extensif au decours d’une diarrhee aigue. Les examens biologiques montraient une anemie, une thrombopenie, une insuffisance renale, une hypergammaglobulinemie, un clone lymphocytaire B circulant minime, une hypoparathyroidie et une hypothyroidie auto-immune. La biopsie cutanee montrait une vascularite avec necrose fibrinoide des petits vaisseaux. Le tocilizumab, associe a des perfusions de methylprednisolone, etait inefficace. Les echanges plasmatiques associes au rituximab ont permis la remission initiale, consolidee par 6 cures de rituximab, cyclophosphamide et dexamethasone. Conclusion Le syndrome TAFRO peut s’associer a une vascularite necrosante cutanee. La resistance possible au tocilizumab merite d’etre connue.

TAFRO syndrome and cutaneous necrotizing vasculitis

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TAFRO syndrome and cutaneous necrotizing vasculitis