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  • • Neutropenia

    • Recurrent infections

    • Splenomegaly

    • Affects patients with seropositive nodular rheumatoid arthritis


  • • 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)


      -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



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

Treatment Monitoring

  • • Neutrophil counts


  • • Infection


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

    • Splenectomy beneficial even if no postoperative increase in neutrophil count


Capsoni F. Sarzi-Puttini P. Zanella A. Primary and secondary autoimmune neutropenia. Arthritis Research & Therapy. 2005, 7(5):208-14.  [PubMed: 16207350]

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