Felty’s syndrome – a rare case of febrile neutropenia

Luís Rodrigues, Glória Nunes da Silva, António Pais de Lacerda


Felty’s syndrome (rheumatoid arthritis with neutropenia and splenomegaly) is a rare condition with poor long-term prognosis, mainly as a result of severe infection risk. An effective treatment strategy has not been developed so far and current treatment options are based upon case reports, small series and clinical experience since no randomized clinical trials are available. The authors describe the case of a 53-year-old female patient with a 14-year history of rheumatoid arthritis presenting with fever, neutropenia and splenomegaly. Broad-spectrum antibiotics and granulocyte colony-stimulating factor were administered with good clinical outcome and low dose methotrexate for disease control was successfully initiated after discharge. We would like to highlight the importance of being aware of this syndrome in the differential diagnosis of long term rheumatoid arthritis patients presenting with febrile neutropenia.


Felty’s syndrome; rheumatoid arthritis; febrile neutropenia

Full Text:



Felty AR. Chronic arthritis in the adult associated with splenomegaly and leucopenia: a report of five cases of an unusual clinical syndrome. Johns Hopkins Hosp Bull 1924; 35:16-20.

Balint GP, Balint PV. Felty’s syndrome. Best Pract Res Clin Rheumatol 2004; 18:631-645.

Xiao RZ, Xiong MJ, Long ZJ, Fan RF, Lin DJ. Diagnois of Felty’s syndrome, distinguished from hematological neoplasm: A case report. Oncol Lett 2014; 7:713-716.

Stanworth SJ, Bhavnani M, Chattopadhya C, Miller H, Swinson DR. Treatment of Felty’s syndrome with the haemopoietic growth factor granulocyte colony-stimulating factor (G-CSF). Q J Med 1998; 91:49-56.

Owlia MB, Newman K, Akhtari M. Felty’s Syndrome, Insights and Updates. Open Rheumatol J 2014; 8:129-136.

Laszlo J, Jones R, Silberman HR, Banks PM. Splenectomy for Felty’s syndrome. Clinicopathological study of 27 patients. Arch Intern Med 1978; 138:597-602.

Ruderman M, Miller LM, Pinals RS. Clinical and serologic observations on 27 patients with Felty’s syndrome. Arthritis Rheum 1968; 11:377-384.

Sienknecht CW, Urowitzz MB, Pruzanski W, Stein HB. Felty’s syndrome. Clinical and serologic analysis of 34 cases. Ann Rheum Dis 1977; 36:500-507.

Campion G, Maddison PJ, Goulding N, et al. The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic asociations. Medicine 1990; 69:69-80.

Burks EJ, Loughran TP Jr. Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome. Blood Rev 2006; 20:245-266.

Breedveld FC, Fibbe WE, Cats A. Neutropenia and infection in Felty’s syndrome. Br J Rheumatol 1988; 27:191-197.

Hellmich B, Csernok E, Schatz H, Gross WL, Schnabel A. Autoantibodies against granulocyte colony-stimulating factor in Felty’s syndrome and neutropenic systemic lupus erythematosus. Arthritis Rheum 2002; 46:2384-2391.

Souza E, Bezerra F, Passos L, Ribeiro S. Felty's syndrome: case report and review of therapeutics. Rev Bras Reumatol 2007; 47-4: 303-308.

DOI: http://dx.doi.org/10.22551/2019.23.0602.10153

Copyright (c) 2019 Luís Rodrigues, Glória Nunes da Silva, António Pais de Lacerda

Creative Commons License
Archive of Clinical Cases is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.


ISSN: 2360-6975