Diagnostic Approach

  • Clinical Evaluation: Detailed history, physical examination, and family history.
  • Laboratory Tests: Immunoglobulin levels, lymphocyte subsets, and functional assays.
  • Genetic Testing: Identifying specific gene mutations.
  • Imaging: Assessing lymphoid tissue and organ involvement.

Treatment Strategies

  • Immunoglobulin Replacement: For antibody deficiencies.
  • Antibiotics and Antifungals: To manage infections.
  • Hematopoietic Stem Cell Transplantation: Curative option for certain PIDs.
  • Targeted Therapies: Modulating immune responses.
  • Supportive Care: Nutritional support, vaccinations, and infection prevention.