PT  - JOURNAL ARTICLE
AU  - Sylvain Raoul Simeni Njonnou
AU  - Justine Deuson
AU  - Claire Royer-Chardon
AU  - Frédéric Alain Vandergheynst
AU  - Virginie De Wilde
TI  - Unexplained cause of thrombocytopenia, fever, anasarca and hypothyroidism: TAFRO syndrome with thrombotic microangiopathy renal histology
AID  - 10.1136/bcr-2019-234155
DP  - 2020 Jun 01
TA  - BMJ Case Reports
PG  - e234155
VI  - 13
IP  - 6
4099  - http://casereports.bmj.com/content/13/6/e234155.short
4100  - http://casereports.bmj.com/content/13/6/e234155.full
SO  - BMJ Case Reports2020 Jun 01; 13
AB  - 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.