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Case report
Unexplained cause of thrombocytopenia, fever, anasarca and hypothyroidism: TAFRO syndrome with thrombotic microangiopathy renal histology
  1. Sylvain Raoul Simeni Njonnou1,2,
  2. Justine Deuson3,
  3. Claire Royer-Chardon4,
  4. Frédéric Alain Vandergheynst1 and
  5. Virginie De Wilde3
  1. 1Internal Medicine, Hopital Erasme, Brussels, Belgium
  2. 2Internal Medicine and Specialties, Faculty of Medicine and Pharmaceutical Sciences, University of Dschang, Dschang, Cameroon
  3. 3Heamatology, Hopital Erasme, Brussels, Belgium
  4. 4Pathology, Hopital Erasme, Brussels, Belgium
  1. Correspondence to Dr Sylvain Raoul Simeni Njonnou; raoulsims{at}yahoo.fr

Abstract

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.

  • immunological products and vaccines
  • haematology (incl blood transfusion)
  • acute renal failure
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Footnotes

  • Contributors Conception and design, acquisition of data, analysis, interpretation of data and follow-up of the patient: SRSN, JD, CR-C, FVDG, VDW. Drafting the article or revising it critically for important intellectual content: SRSN, JD, CR-C, FVDG, VDW. Final approval: all authors.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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