TAFRO syndrome with skin manifestations treated with bortezomib and tocilizumab; a case report

Abstract

TAFRO syndrome is a new presentation of idiopathic multicentric Castleman disease which is termed as thrombocytopenia, anasarca, myelofibrosis, renal failure and organomegaly (TAFRO). The exact pathophysiology of TAFRO syndrome is unclear and management is mostly based on case reports and expert opinion. In this report, a 37 years old male patient with TAFRO syndrome is discussed. The patient was referred with fever, sweating, anorexia, abdominal distension and generalized edema which has been hospitalized multiple times for such complaints. The patient also developed skin lesions dispersed in red nodules, which was reported as "granuloid hemangioma". Renal biopsy suggested mesangioproliferative glomerulonephritis and bone marrow specimen showed hypercellular active marrow with reticulin fibrosis. The lymph node biopsies were reported as Castleman disease. This report demonstrates that different manifestations of TAFRO syndrome may overlap with other syndromes and can be managed by Bortezomib and Tocilizumab.

TAFRO syndrome: A disease that known is half cured

“…Its therapeutic mechanism is specifically to inhibit the activity of the proteasome 26S subunit, to block the degradation of IκB and to prevent the release of IL-6, TNF-α, and IL-1β. Recently, two patients with TAFRO syndrome in China and Iran were treated with bortezomib, 81,82 and the desired therapeutic effect was obtained.…”