- Severe subset of seropositive rheumatoid arthritis complicated by granulocytopenia and splenomegaly
- Splenectomy is indicated
- Granulocytopenia and nonhealing leg ulcers
- Recurrent or severe infections (pneumonia or septicemia)
- Despite nonpharmacologic efforts to reduce risk of infection and use of medical therapies, including the conventional synthetic, biologic, and targeted synthetic disease-modifying antirheumatic drugs (DMARDs) and granulocyte colony-stimulating factor (G-CSF).
- Patients with recurrent or severe infections usually have severe granulocytopenia (<1000 cells/microL)