Felty syndrome
Updates to Article Attributes
Body
was changed:
Felty syndrome is a rare haematological syndrome in rheumatoid arthritis.
Epidemiology
It is thought to occur in ~ 1% of patients with rheumatoid arthritis 2.
Clinical presentation
Felty syndrome comprises of the triad of:
-
rheumatoid arthritis
- typically with severe articular and extra-articular disease
- splenomegaly
- neutropenia
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 5
- antinuclear antibody (ANA): 47-100% are positive 5
- HLA-DR4*0401 antigen: 78% of patients have the antigen 5
- large granular lymphocyte (LGL) expansion: ~ 30% of patients may have it 5
Treatment and prognosis
Treatment is through immunosuppression to treat Felty syndrome and rheumatoid arthritis, such as use of methotrexate and rituximab 6. If frequent severe bacterial infections, G-CSF may be administered 6.
History and etymology
It is named after Augustus Roi Felty (1895 -1964), an American physician 1.
Differential diagnosis
-<a title="large granular lymphocyte leukaemia" href="/articles/large-granular-lymphocyte-leukaemia">large granular lymphocyte (LGL) leukaemia</a> <sup>6</sup>- +<a href="/articles/large-granular-lymphocyte-leukaemia">large granular lymphocyte (LGL) leukaemia</a> <sup>6</sup>
Unable to process the form. Check for errors and try again.