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
. 2023 Oct 17:10:1238405.
doi: 10.3389/fmed.2023.1238405. eCollection 2023.

Felty's syndrome

Christoph Wegscheider  1   2 , Vera Ferincz  1   2 , Karin Schöls  1   2 , Andreas Maieron  1   2
Affiliations
Review

Felty's syndrome

Christoph Wegscheider et al. Front Med (Lausanne). .

Abstract

Felty's syndrome was first described in 1924 by the US-American physician Augustus Roi Felty as a triad of rheumatoid arthritis, splenomegaly and leucopenia. Even nearly 100 years later, this rare syndrome is still paralleled by diagnostic and therapeutic challenges and its pathogenesis is incompletely understood. Neutropenia with potentially life-threatening infections is the main problem and several pathomechanisms like Fas-mediated apoptosis, anti-neutrophil antibodies, anti-G-CSF antibodies, neutrophil consumption in the context of NETosis and suppression of granulopoiesis by T-LGLs have been suggested. Felty's syndrome has various differential diagnoses as splenomegaly and cytopenia are common features of different infectious diseases, malignancies and autoimmune disorders. Additionally, benign clonal T-/NK-LGL lymphocytosis is increasingly noticed in Felty's syndrome, which further complicates diagnosis. Today's treatment options are still sparse and are largely based on case reports and small case series. Methotrexate is the mainstay of therapy, followed by rituximab, but there is less evidence for alternatives in the case of adverse reactions or failure of these drugs. This article gives an updated review about Felty's syndrome including its pathogenesis and treatment options.

Keywords: Felty syndrome; JAK inhibitor; T-LGL; rheumatoid arthritis; rituximab.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Stepwise approach for Felty’s syndrome treatment.
See this image and copyright information in PMC

References

    1. Oshimi K. Clinical features, pathogenesis, and treatment of large granular lymphocyte leukemias. Intern Med. (2017) 56:1759–69. doi: 10.2169/internalmedicine.56.8881 - DOI - PMC - PubMed
    1. Prochorec-Sobieszek M, Rymkiewicz G, Makuch-Łasica H, Majewski M, Michalak K, Rupiński R, et al. . Characteristics of T-cell large granular lymphocyte proliferations associated with neutropenia and inflammatory arthropathy. Arthritis Res Ther. (2008) 10:R55. doi: 10.1186/ar2424, PMID: - DOI - PMC - PubMed
    1. Mariotti B, Calabretto G, Rossato M, Teramo A, Castellucci M, Barilà G, et al. . Identification of a miR-146b-Fas ligand axis in the development of neutropenia in T large granular lymphocyte leukemia. Haematologica. (2020) 105:1351–60. doi: 10.3324/haematol.2019.225060, PMID: - DOI - PMC - PubMed
    1. Savola P, Brück O, Olson T, Kelkka T, Kauppi MJ, Kovanen PE, et al. . Somatic STAT3 mutations in Felty syndrome: an implication for a common pathogenesis with large granular lymphocyte leukemia. Haematologica. (2018) 103:304–12. doi: 10.3324/haematol.2017.175729, PMID: - DOI - PMC - PubMed
    1. Gorodetskiy V, Sidorova Y, Biderman B, Ryzhikova N, Vasilyev V, Sudarikov A, et al. . Prevalence of STAT3 mutations in patients with rheumatoid arthritis-associated T-cell large granular lymphocytic leukaemia and Felty syndrome STAT3 mutations in RA-associated T-LGL leukaemia and FS/V. Gorodetskiy et al. Clin Exp Rheumatol. (2022). doi: 10.55563/clinexprheumatol/dyi9hd - DOI - PubMed