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  • • Neutropenia

    • Recurrent infections

    • Splenomegaly

    • Affects patients with seropositive nodular rheumatoid arthritis

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Epidemiology

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  • • 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

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Symptoms and Signs

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  • • Recurring infections

    • Splenomegaly

    • Chronic leg ulcers

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Laboratory Findings

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  • • Decreased neutrophil count

    • Increased granulopoiesis in the bone marrow

    • High levels of IgG on the surface of neutrophils

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  • • 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

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Rule Out

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  • • Other causes of neutropenia

    • -Aplastic anemia

      -Pure white cell aplasia

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

      -Sepsis

      -Immune mediated

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  • • CBC

    • Neutrophil count

    • Antineutrophil surface IgG

    • Bone marrow biopsy

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When to Admit

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  • • Severe neutropenia

    • Infectious complications

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When to Refer

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

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  • • Splenectomy removes source of antibody-mediated neutrophil destruction

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Surgery

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Indications

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

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Treatment Monitoring

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  • • Neutrophil counts

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Complications

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  • • Infection

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Prognosis

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

    • Splenectomy beneficial even if no postoperative increase in neutrophil count

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References

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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