Felty Syndrome

• • Neutropenia

  • Recurrent infections

  • Splenomegaly

  • Affects patients with seropositive nodular rheumatoid arthritis

Epidemiology

• • Affects approximately 1% of patients with rheumatoid arthritis

  • High levels of IgG on the surface of neutrophils with evidence of increased granulopoiesis in the bone marrow

  • Recurrent infections are due to decreased and dysfunctional neutrophils coated with IgG

Symptoms and Signs

• • Recurring infections

  • Splenomegaly

  • Chronic leg ulcers

Laboratory Findings

• • Decreased neutrophil count

  • Increased granulopoiesis in the bone marrow

  • High levels of IgG on the surface of neutrophils

• • Pathologic analysis of the spleen in patients with Felty syndrome shows a larger proportionate increase in the white pulp as opposed to most conditions of splenomegaly

  • There is evidence of excess accumulation of neutrophils in both the T cell zone of the white pulp as well as the cord and sinuses of the red pulp

Rule Out

• • Other causes of neutropenia

  • -Aplastic anemia

    -Pure white cell aplasia

    -Drugs (sulfonamides, procainamide, penicillin, cyclosporines, cimetidine, phenytoin, chlorpropamide)

    -Sepsis

    -Immune mediated

• • CBC

  • Neutrophil count

  • Antineutrophil surface IgG

  • Bone marrow biopsy

When to Admit

• • Severe neutropenia

  • Infectious complications

When to Refer

• • All patients should be managed in conjunction with an hematologist

• • Splenectomy removes source of antibody-mediated neutrophil destruction

Surgery

Indications

• • Patients with recurrent bacterial infections and evidence of IgG on the surface of neutrophils

Treatment Monitoring

• • Neutrophil counts

Complications

• • Infection

Prognosis

• • Neutropenia will improve in 60-70% after splenectomy; recurrence possible.

  • Splenectomy beneficial even if no postoperative increase in neutrophil count

References