Felty syndrome

Felty syndrome is a rare hematological syndrome in rheumatoid arthritis.

Epidemiology

It is thought to occur in ~ 1% of patients with rheumatoid arthritis .

Clinical presentation

Felty syndrome comprises of the triad of:

Additionally, patients may have bicytopaenia or pancytopaenia, recurrent bacterial infections, and non-cirrhotic portal hypertension.

Pathology

Serological markers
  • rheumatoid factor (RF): >95% of patients are positive
  • antinuclear antibody (ANA): 47-100% are positive
  • HLA-DR4*0401 antigen: 78% of patients have the antigen
  • large granular lymphocyte (LGL) expansion: ~ 30% of patients may have it

​Treatment and prognosis

Treatment is through immunosuppression to treat Felty syndrome and rheumatoid arthritis, such as use of methotrexate and rituximab . If frequent severe bacterial infections, G-CSF may be administered .

History and etymology

It is named after Augustus Roi Felty (1895 -1964), an American physician .

Differential diagnosis

  • large granular lymphocyte (LGL) leukemia