Diagnosis of autoimmune neutropenia (AIN) in infants is important, because it allows the exclusion of more severe forms of neutropenia that have an increased risk for leukemia. AIN is characterized by chronic neutropenia, which spontaneously resolves within several months to a few years, and mild infections. Diagnosis is confirmed by the presence of antibodies directed against neutrophil antigens. The human neutrophil antigen (HNA) system is a polymorphic system, which includes five antigen groups with different polymorphisms. In AIN, antibodies are mostly directed against HNA-1 (or against a specific allele of HNA-1) and HNA-4. Here, we present a series of 116 infants with AIN. We observed that anti-neutrophil antibodies were present in 60% cases; directed against HNA-1a in 73% of cases. In addition, we showed there was a bias in the HNA allele distribution in these infants because the frequency of the HNA-1a allele was greater in comparison with controls.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1111/j.1399-3038.2010.01117.x | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
An Unusual Case of Overlapping Immunoglobulin G4-Related Disease and Systemic Lupus Erythematosus.
Cureus
December 2024
Nephrology, Unidade Local de Saúde de São José, Lisbon, PRT.
Immunoglobulin G4-related disease (IgG4-RD) and systemic lupus erythematosus (SLE) are multisystemic autoimmune disorders that can present with renal manifestations. Overlapping cases of these diseases are extremely rare and present both diagnostic and therapeutic challenges. We report the case of a 70-year-old male with a history of autoimmune pancreatitis, who was admitted with fatigue, weight loss, and worsening kidney function.
View Article and Find Full Text PDFExploring the landscape of congenital and idiopathic neutropenia in Moroccan children: a comprehensive retrospective analysis.
Immunol Res
January 2025
Laboratory of Clinical Immunology, Infectiology, and Autoimmunity (LICIA), Faculty of Medicine and Pharmacy of Casablanca, Hassan II University, Casablanca, Morocco.
Congenital neutropenia (CoN) is a heterogeneous group of inborn errors of immunity (IEI) characterized by recurrent infections and early onset of neutropenia (NP). This study aimed to investigate the demographic and clinical data of children with CoN and idiopathic neutropenia (IN) in Morocco. We performed a retrospective study of patients with CoN and analyzed the clinical and laboratory findings of patients with CoN and IN diagnosed between 1999 and 2018 in a clinical immunology unit of a large pediatric hospital.
View Article and Find Full Text PDFTransient suppression of high-prevalence Kell blood group antigens and concomitant development of a Kell-related antibody that appears to recognize a high-prevalence Kell antigen not previously defined.
Immunohematology
December 2024
International Blood Group Reference Laboratory, NHS Blood and Transplant, Bristol, UK.
A previously healthy 32-year-old male patient was admitted to hospital with malaise, dyspnea, anemia, thrombocytopenia, and leukopenia. Anemia and thrombocytopenia worsened during the third week. Considering the possible need for transfusion, routine ABO and D typing and an antibody detection test were performed.
View Article and Find Full Text PDFPulmonary and Medullary Tuberculosis: An Uncommon Cause of Evans Syndrome in Adults.
Cureus
November 2024
Hematology, Cheikh Khalifa International University Hospital, Mohammed VI University of Sciences and Health, Casablanca, MAR.
Evans syndrome (ES) is a rare syndrome characterised by the association of autoimmune idiopathic hemolytic anemia (AIHA) with immune thrombocytopenia (ITP) and, less commonly, autoimmune neutropenia (AIN). ES may be primary or secondary to some aetiology, including, exceptionally, tuberculosis. We describe a case of association between pulmonary and medullary tuberculosis and Evans syndrome with an effective response to antitubercular treatment and corticosteroids.
View Article and Find Full Text PDFClinical correlations of serum anti-dsDNA immunoglobulin subfamilies in patients with systemic lupus erythematosus (SLE).
Autoimmunity
December 2025
Department of Clinical Immunology & Allergy, Westmead Hospital & ICPMR, Westmead, NSW, Australia.
Systemic lupus erythematosus (SLE) is an extremely heterogenous autoimmune disorder. A key biomarker, the double stranded (ds) DNA autoantibody, provides diagnostic specificity for SLE. We analyzed anti-dsDNA by mass spectrometry (MS) to determine if ascertaining the autoantibody's heavy chain variable region (IGHV) may hold any clinical relevance.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!