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:
- 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
- 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
Siehe auch:
Assoziationen und Differentialdiagnosen zu Felty syndrome:
