Conference abstract

That´s about a frankly rare and obscure diagnosis: an exceptional case of TAFRO

Pan African Medical Journal - Conference Proceedings. 2023:16(37).15 Mar 2023.
doi: 10.11604/pamj-cp.2023.16.37.2309
Archived on: 15 Mar 2023
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Keywords: TAFRO syndrome, kidney biopsy, thrombotic microangiopathy, rituximab, tocilizumab

That´s about a frankly rare and obscure diagnosis: an exceptional case of TAFRO

Sylvain Raoul Simeni Njonnou1,2,&, Justine Deuson3, Claire Royer-Chardon4, Frédéric Alain Vandergheynst1, Virginie De Wilde3

1Internal Medicine, Hospital Erasme, Brussels, Belgium, 2Internal Medicine and Specialties, Faculty of Medicine and Pharmaceutical Sciences, University of Dschang, Dschang, Cameroon, 3Hematology, Hospital Erasme, Brussels, Belgium, 4Pathology, Hospital Erasme, Brussels, Belgium

&Corresponding author

Introduction: TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis or renal dysfunction and organomegaly) syndrome is a systemic inflammatory disease characterized 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.

Methods: 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.

Results: kidney biopsy showed double contours of the glomerular basement membrane, mesangiolysis, and endothelial swelling compatible with thrombotic microangiopathy (TMA) and TAFRO syndrome. Corticosteroids, tocilizumab, and rituximab successfully treated her.

Conclusion: 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 with severe hypothyroidism, the presence of TMA-like lesions on kidney biopsy, and the treatment by the association of steroids, tocilizumab, and rituximab.