TAFRO syndrome

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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]

Contents

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]

Cause

In 2025 Japanese researchers reported that a young man with no underlying medical condition was diagnosed with TAFRO syndrome five days after receiving his second dose of COVID mRNA vaccine (mRNA1273). The patient was admitted with typical symptoms of the syndrome, and elevated levels of interleukin-6 (110.0 pg/ml) and vascular endothelial growth factor (480 pg/ml) were found in laboratory tests. Their findings supported earlier research and suggested that TAFRO syndrome is linked to mRNA vaccine as it could trigger the mechanism to activate mechanistic target of rapamycin (mTOR) signalling. [7]

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. 1 2 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.
  7. ↑ Aizawa, Y. et. al. (2025). "Thrombocytopenia, anasarca, fever, reticulum fibrosis, and organomegaly syndrome triggered by vaccination against SASRS-CoV-2 messenger RNA vaccine". Modern Rheumatology Case Reports. 9. doi:10.1093/mrcr/rxaf042.
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