Unexplained cause of thrombocytopenia, fever, anasarca and hypothyroidism: TAFRO syndrome with thrombotic microangiopathy renal histology.

TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis or renal dysfunction and organomegaly) syndrome is a systemic inflammatory disease characterised by thrombocytopenia, anasarca, fever or inflammatory syndrome, reticulin myelofibrosis or renal dysfunction and organomegaly. It was first described as a subtype of idiopathic multicentric Castleman disease. Here, we report the case of a 42-year-old woman presenting with thrombocytopenia, anasarca, inflammatory syndrome, renal insufficiency, reticulin myelofibrosis at bone marrow biopsy and cervical and axillary lymph nodes. Kidney biopsy showed double contours of the glomerular basement membrane, mesangiolysis and endothelial swelling compatible with thrombotic microangiopathy (TMA) as well as with TAFRO syndrome. She was successfully treated by corticosteroids, tocilizumab and rituximab. This new case description of TAFRO syndrome underlines three features of this disease rarely described in the literature and never simultaneously in the same patient: the association to severe hypothyroidism, the presence of TMA-like lesions on kidney biopsy and the treatment by the association of steroids, tocilizumab and rituximab.

Simeni Njonnou SR ，Deuson J ，Royer-Chardon C ，Vandergheynst FA ，Wilde V ... -  《-》

Kidney biopsy findings in two patients with TAFRO syndrome: case presentations and review of the literature.

Zhou Q ，Zhang Y ，Zhou G ，Zhu J ... -  《BMC Nephrology》

The first case of thrombocytopenia, anasarca, fever, renal impairment or reticulin fibrosis, and organomegaly (TAFRO) syndrome with unilateral adrenal necrosis: a case report.

Fujiwara Y ，Ito K ，Takamura A ，Nagata K ... -  《-》

Thrombotic microangiopathy on kidney biopsy in a patient with TAFRO syndrome.

Ozeki T ，Tsuji M ，Yamamoto J ，Shigematsu C ，Maruyama S ... -  《-》

Renal Pathologic Findings in TAFRO Syndrome: Is There a Continuum Between Thrombotic Microangiopathy and Membranoproliferative Glomerulonephritis? A Case Report and Literature Review.

Leurs A ，Gnemmi V ，Lionet A ，Renaud L ，Gibier JB ，Copin MC ，Hachulla E ，Hatron PY ，Launay D ，Fajgenbaum D ，Terriou L ... -  《Frontiers in Immunology》