Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2024 Oct 9;46(10):11255-11269.
doi: 10.3390/cimb46100668.

Pathophysiology, Treatment, and Prognosis of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome: A Review

Takuya Kakutani  1 , Riko Kamada  1 , Yotaro Tamai  2
Affiliations
Review

Pathophysiology, Treatment, and Prognosis of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome: A Review

Takuya Kakutani et al. Curr Issues Mol Biol. .

Abstract

TAFRO syndrome, first reported in 2010, is a systemic inflammatory disease with a rapid onset and potentially fatal course if not treated promptly and appropriately. The name is derived from the initial letters describing the characteristic symptoms of thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It is sometimes considered a special subtype of idiopathic multicentric Castleman disease (iMCD) because lymph node biopsies often reveal the pathology findings seen in iMCD. However, its clinical manifestations and prognoses are not well documented. Since the clinical manifestations and prognoses of TAFRO syndrome differ significantly from those of iMCD, it is recognized as an independent disease concept and considered to partially overlap with the pathology of MCD. The pathogenesis of TAFRO syndrome remains largely unknown. Due to the lack of appropriate treatment, it often presents with multiple organ dysfunction and fatality. In this review, we summarized new findings on the pathogenesis of TAFRO syndrome and discussed current effective therapies and future treatment strategies.

Keywords: IL-6; JAK-STAT; PI3K/Akt/mTOR; TAFRO syndrome; type 1 interferon.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Classification of MCD and TAFRO syndrome. iMCD-TAFRO syndrome is classified as a combination of cases that present with symptoms similar to TAFRO syndrome and have histological evaluations that reveal MCD-like findings and cases that do not undergo histological evaluations and exclude other diseases similar to TAFRO syndrome.
Figure 2
Figure 2
The 2015 disease severity classification for TAFRO syndrome. Minor update added in 2019.
Figure 3
Figure 3
Diagnostic criteria for TAFRO syndrome created in 2021.
Figure 4
Figure 4
Diagnostic algorithm for TAFRO syndrome.
Figure 5
Figure 5
Pathophysiology and potential therapeutic targets in TAFRO syndrome (other than IL-6 pathway).
Figure 6
Figure 6
IL-6 signaling pathway and therapeutic targets.
See this image and copyright information in PMC

References

    1. Takai K., Nikkuni K., Shibuya H., Hashidate H. [Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly] Rinsho Ketsueki. 2010;51:320–325. - PubMed
    1. Inoue M., Ankou M., Hua J., Iwaki Y., Hagihara M., Ota Y. Complete resolution of TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly) after immunosuppressive therapies using corticosteroids and cyclosporin A: A case report. J. Clin. Exp. Hematop. 2013;53:95–99. doi: 10.3960/jslrt.53.95. - DOI - PubMed
    1. Iwaki N., Sato Y., Takata K., Kondo E., Ohno K., Takeuchi M., Orita Y., Nakao S., Yoshino T. Atypical hyaline vascular-type Castleman’s disease with thrombocytopenia, anasarca, fever, and systemic lymphadenopathy. J. Clin. Exp. Hematop. 2013;53:87–93. doi: 10.3960/jslrt.53.87. - DOI - PubMed
    1. Masaki Y., Nakajima A., Iwao H., Kurose N., Sato T., Nakamura T., Miki M., Sakai T., Kawanami T., Sawaki T., et al. Japanese variant of multicentric Castleman’s disease associated with serositis and thrombocytopenia—A report of two cases: Is TAFRO syndrome (Castleman- Kojima disease) a distinct clinicopathological entity? J. Clin. Exp. Hematop. 2013;53:79–85. doi: 10.3960/jslrt.53.79. - DOI - PubMed
    1. Louis C., Vijgen S., Samii K., Chalandon Y., Terriou L., Launay D., Fajgenbaum D.C., Seebach J.D., Muller Y.D. TAFRO syndrome in Caucasians: A case report and review of the literature. Front. Med. 2017;4:149. doi: 10.3389/fmed.2017.00149. - DOI - PMC - PubMed

LinkOut - more resources