Selective IgM deficiency: Follow-up and outcome.

Area of Science:

• Immunology
• Pediatric Immunology

Background:

• Selective IgM deficiency (sIgMD) is a primary immunodeficiency (PID).
• Understanding its clinical and immunological spectrum is essential for patient management.

Purpose of the Study:

• To define the clinical and immunological features of selective IgM deficiency (sIgMD).

Main Methods:

• Retrospective analysis of medical records of pediatric patients diagnosed with sIgMD.
• Inclusion criteria based on established diagnostic criteria for sIgMD.

Main Results:

• Fifteen (45.4%) patients experienced infections (respiratory, skin).
• Six (18%) had chromosomal anomalies/syndromes, and six (18%) had autoimmune/inflammatory diseases.
• Three (9%) developed malignancies; 24.2% were asymptomatic.
• A significant proportion were later diagnosed with specific PIDs.

Conclusions:

• sIgMD can be associated with genetic disorders, autoimmune/inflammatory conditions, and allergic diseases.
• Increased malignancy risk is observed in patients with sIgMD.
• Regular monitoring in specialized immunology clinics is recommended for early detection and management.