Felty's syndrome

Introduction

Triad of rheumatoid arthritis (RA), hypersplenism & leukopenia.

Epidemiology

• occurs in < 1% of patients with RA
• 2/3 of patients are women
• mean duration of RA prior to onset of Felty's syndrome is 15 years

Clinical manifestations

• Felty's triad of rheumatoid arthritis, splenomegaly, neutropenia
  • erosive polyarthritis (RA)
  • leukopenia (neutropenia)
  • hypersplenism, splenomegaly
• other manifestations may be present
  • recurrent fever with & without apparent infection
  • rheumatoid nodules are common
  • weight loss
  • lymphadenopathy
  • skin hyperpigmentation
  • cutaneous ulcers in lower extremities
  • vasculitis
  • peripheral neuropathy
  • keratoconjunctivitis sicca
  • xerostomia
  • pleuritis

Laboratory

• complete blood count (CBC)
  • leukopenia
    • profound neutropenia < 500/mm3 is rare
    • lymphopenia in 1/3 of patients
  • anemia
  • thrombocytopenia (40%)
• peripheral blood smear
  • large granular lymphocytes in 40%
• serology
  • high titers of rheumatoid factor (RF)
  • antinuclear antibody (ANA) in 2/3 of patients
  • decreased complement components (CH50) occurs with vasculitis
  • HLA-DR4(+)
• bone marrow biopsy
  • myeloid hyperplasia with maturation arrest
  • marrow is rarely hypocellular

Complications

• bacterial infections, especially of the skin & lungs
• patients commonly die of sepsis, despite aggressive therapy
• large granular lymphocyte syndrome

Management

• corticosteroids
• methotrexate
• lithium carbonate
• granulocyte colony-stimulating factor (G-CSF)
• splenectomy (except LGLS)
  • mitigates hematologic abnormalities, recurrent bacterial infections & leg ulcerations
  • does NOT affect overall course of rheumatoid arthritis

More general terms

• syndrome
• rheumatoid arthritis (RA)

More specific terms

• large granular lymphocyte syndrome (LGLS, LGL)

Patient information

Felty's syndrome patient information