II. Epidemiology
- More common in females by 8:1 ratio
III. Causes: Acute Autoimmune Neutropenia
- Primary Autoimmune Neutropenia
- Peak Prevalence in children age <10 years
- Most common in girls
- Typically self limited
- Secondary Causes
- Drug-Induced Autoimmune Conditions
- Viral Infections
IV. Types: Chronic Autoimmune Neutropenia
- Primary Autoimmune Neutropenia
- Neutropenia >3 months
- Associated with minor infections
- ANC increases during times of infection or emotional stress
- Secondary Causes
- Autoimmune disorders
- Rheumatoid Arthritis (RA)
- Systemic Lupus Erythematosus
- Sjogren Synrome
- Felty Syndrome
- Hematologic Malignancy
- Chronic Lymphocytic Leukemia
- Hodgkin Lymphoma
- Non-Hodgkin Lymphoma
- Hairy Cell Leukemia
- Waldenstrom Macroglobulinemia
- T-Cell Large Granular LymphocyteLeukemia
- Associated with Felty Syndrome (advanced RA, Neutropenia, Splenomegaly)
- Solid cancers (e.g. thymoma)
- Immunodeficiency (e.g. Common Variable Immunodeficiency, autoimmune lymphoproliferative syndrome)
- Autoimmune disorders
V. Labs
- Antineutrophil Antibody positive
- Low Test Sensitivity and Test Specificity
- Consider at least 2 separate tests
- Does not predict course
-
Bone Marrow Biopsy indications
- Clinical suspicion high, but negative Antineutrophil Antibody
VI. Management
- See Neutropenia
- Treat underlying causes
- Consider Granulocyte Colony Stimulating Factor (G-CSF, esp. with severe recurrent infections)