TAFRO syndrome

TAFRO syndrome is a rare human systemic disease. The name is formed from the initials of thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly.[1][2][3] It was first described, in three patients, in 2010 by Takei et al,[4] and was discussed at two meetings held in 2012.[5]

It has "significant overlap" with Castleman's disease but "[its] features warrant its classification as a separate subtype of idiopathic multicentric Castleman's disease (iMCD)".[3]

It was reported in 2025 that Anakinra had been used successfully in two cases of paediatric TAFRO syndrome.[6]

References

  1. ^ Masaki, Y; Ueda, Y; et al. (1 January 2023). "TAFRO Syndrome: A Disease Requiring Immediate Medical Attention". Internal Medicine (Tokyo, Japan). 62 (1): 27–32. doi:10.2169/internalmedicine.9622-22. PMC 9876714. PMID 35598998.
  2. ^ Katsuhiro, Miura; Haruna, Nishimaki-Watanabe; et al. (June 2024). "TAFRO Syndrome: Guidance for Managing Patients Presenting Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, Renal Insufficiency, and Organomegaly". Biomedicines. 12 (6). doi:10.3390/biomedicine (inactive 1 July 2025). ISSN 2227-9059.{{cite journal}}: CS1 maint: DOI inactive as of July 2025 (link)
  3. ^ a b Caballero, JC; Conejero, N; et al. (13 May 2024). "Unraveling TAFRO Syndrome: An In-Depth Look at the Pathophysiology, Management, and Future Perspectives". Biomedicines. 12 (5): 1076. doi:10.3390/biomedicines12051076. PMC 11118735. PMID 38791038.
  4. ^ Takai, K; Nikkuni, K; et al. (May 2010). "[Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly]". [Rinsho ketsueki] The Japanese journal of clinical hematology. 51 (5): 320–5. PMID 20534952.
  5. ^ Kawabata, H; Takai, K; et al. (2013). "Castleman-Kojima disease (TAFRO syndrome): a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly: a status report and summary of Fukushima (6 June, 2012) and Nagoya meetings (22 September, 2012)". Journal of Clinical and Experimental Hematopathology. 53 (1): 57–61. doi:10.3960/jslrt.53.57. PMID 23801135.
  6. ^ Palmeri, Serena; Ferro, Jacopo; et al. (15 May 2025). "Efficacy of High-Dose Intravenous Anakinra in Pediatric TAFRO Syndrome: Report of Two Cases and Literature Review". Pediatric Blood & Cancer. 72 (8): e31759. doi:10.1002/pbc.31759. PMID 40372261.
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