SHM Converge Scientific Abstract ePoster Gallery-Primary Autoimmune Neutropenia: Case Report

Pdf Summary

This case describes a 36-year-old woman with fever, chills, neck tenderness, and aphthous ulcers who was found to have profound neutropenia with an ANC of 0. Although she had recently received clindamycin for streptococcal pharyngitis, review of her history showed intermittent neutropenia over the prior four years, suggesting a chronic process rather than a simple drug reaction.<br /><br />She was initially treated empirically with antibiotics because she met SIRS criteria. A broad evaluation for common causes of neutropenia was negative, including infectious testing, autoimmune markers, nutritional deficiencies (B12, copper, folate), bone marrow biopsy, chromosomal analysis, and myeloid next-generation sequencing. An anti-neutrophil antibody test later confirmed primary autoimmune neutropenia (AIN).<br /><br />Primary AIN is a rare cause of adult neutropenia, most often seen in women. It results from anti-neutrophil antibodies and can look clinically identical to other causes of neutropenia, making diagnosis difficult. Adults with recurrent or prolonged neutropenia without an obvious cause should be evaluated further once common etiologies are excluded.<br /><br />The patient was treated with high-dose oral prednisone followed by a taper for four weeks, leading to normalization of her ANC within one month. Granulocyte colony-stimulating factor was not used because bone marrow biopsy showed adequate reserve. Rituximab was noted as a possible future option, as it is emerging as a treatment for primary autoimmune cytopenias.<br /><br />Overall, this case highlights the importance of considering primary autoimmune neutropenia in adults with unexplained recurrent neutropenia and demonstrates that corticosteroids can be an effective therapy.

Asset Subtitle

Neha Saini

Meta Tag

Author List Maxwell Bressman, Neha Saini

Category Clinical Vignettes

Concept Neutropenia

Concept Primary Autoimmune Neutropenia

Concept Autoimmune Neutropenia

Concept Idiosyncratic Drug-Induced Neutropenia

Concept G-CSF

Distinguished Non-Finalist

Presenter Organization Montefiore Medical Center

Presenting Author Neha Saini

Track Adult

Keywords

primary autoimmune neutropenia

adult neutropenia

profound neutropenia

anti-neutrophil antibodies

neutropenia diagnosis

corticosteroid therapy

prednisone taper

bone marrow biopsy

febrile neutropenia

autoimmune cytopenia

Neutropenia

Primary Autoimmune Neutropenia

Autoimmune Neutropenia

Idiosyncratic Drug-Induced Neutropenia

G-CSF