Year-end Sale % OFF 
Abstract
Antineutrophil antibodies are well recognized causes of neutropenia, producing both quantitative and qualitative defects in neutrophils and increased risk for infection. In primary autoimmune neutropenia (AIN) of infancy, a moderate to severe neutropenia is the sole abnormality; it is rarely associated with serious infections and exhibits a self-limited course. Chronic idiopathic neutropenia of adults is characterized by occurrence in late childhood or adulthood, greater prevalence among females than among males, and rare spontaneous remission. Secondary AIN is more commonly seen in adults and underlying causes include collagen disorders, drugs, viruses and lymphoproliferative disorders. In most patients with AIN, antibodies recognize antigens located on the IgG Fc receptor type 3b but other target antigens have been recently identified in secondary AIN. Granulocyte colony-stimulating factor is a proven treatment in patients with AIN of all types and is now preferred to other possible therapies.
Highlights
The term ‘neutropenia’ is used to define a condition in which circulating neutrophils number less than 1500/μl
autoimmune neutropenia (AIN) are rare disorders in which autoantibodies directed against membrane antigens of neutrophils causes their peripheral destruction
In primary forms of AIN autoantibody specificity has been defined, and usually autoantibodies recognize antigens located on the FcγRIIIb
Summary
The term ‘neutropenia’ is used to define a condition in which circulating neutrophils number less than 1500/μl. Steroids, used in small case series in the past, appear to have limited effect on immune-mediated neutropenias, there are reports of activity in primary and secondary autoimmune forms [13] They seem to work by blocking the reticuloendothelial system and by reducing the formation of autoantibodies. The long-term adverse effects of G-CSF (reduction in myeloid precursors, formation of anti-G-CSF antibodies, osteopenia; for review, see Maheshwari and coworkers [9]) do not normally affect patients with primary AIN, which is usually benign and self-limited, but they must be borne in mind in more severe forms of immune-mediated neutropenia, which require lengthy treatment. In secondary AIN antineutrophil antibodies are usually only one of the causes of the neutropenia, which may be associated (depending on the case) with peripheral sequestration, bone marrow inhibition, or apoptosis (see below). Like in Felty’s syndrome, in patients with SLE and neutropenia G-CSF should preferably only be used in selected cases, at the lowest dose that achieves a circulating neutrophil count of at least 1000/μl [52]
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
