Work

Overlapping autoimmunity and immunodeficiency: a case of selective IgA deficiency with autoimmune hemolytic anemia

Year: 2026

Type: article

Abstract: BACKGROUND: The most prevalent primary immunodeficiency is selective immunoglobulin A deficiency (SIgAD), which is characterized by serum IgA levels equal to or less than 7 mg/dL with normal levels of IgG and IgM in a patient who is at least 4 years old. Most patients are asymptomatic; however, SIgAD increases the risk of respiratory and gastrointestinal infections, autoimmune disorders, transfusion reactions, and atopy. CASE PRESENTATION: Our 4-year-old female patient had a history of recurrent severe infections requiring multiple hospital admissions, raising our suspicion of an immunodeficiency, especially with a family history of SIgAD. She had several episodes of pallor and jaundice that were caused by autoimmune hemolytic anemia, with the last episode being associated with a severe respiratory infection. CONCLUSION: This case highlights the importance of early diagnosis and ongoing follow-up of children with SIgAD to ensure prompt treatment of infections and autoimmune complications even before the age of four years. It also recommends regular monitoring and early supportive intervention to reduce disease flare-ups and monitor for progression to common variable immunodeficiency.

Source: BMC Pediatrics

DOI: 10.1186/s12887-026-06570-7

Authors: Laith Khalaf, Mohammad Hamdan, Meera Lahlouh, Kareem Abdul-Haleem

Institution: An-Najah National University

Language: English

Topic: Immunodeficiency and Autoimmune Disorders

Subfield: Immunology

Field: Immunology and Microbiology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $3,090