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Table 3 Strategies for the treatment and management of PIDs

From: Primary immunodeficiency

  Supportive Definitive
CIDs/SCID Ig replacement therapy (IV or SC) Antibiotic prophylaxis Antifungal prophylaxis Aggressive management of established infections Infectious precautions when hospitalized Withhold all live vaccines BMT HSCT Gene therapy a possibility for some SCIDs
  1. Ig immunoglobulin, IV intravenous, SC subcutaneous, CID combined immunodeficiency, SCID severe combined immunodeficiency, IFNγ interferon-γ, BMT bone marrow transplantation, CGD chronic granulomatous disease, HSCT hematopoietic stem cell transplantation
B-cell disorders Ig replacement therapy (IV or SC) Antibiotic prophylaxis Antifungal prophylaxis depending upon etiology Hearing assessment Assessment of pulmonary status and function Close monitoring for co-morbidities Gene therapy is a potential future treatment in some patients
  1. Ig immunoglobulin, IV intravenous, SC subcutaneous, CID combined immunodeficiency, SCID severe combined immunodeficiency, IFNγ interferon-γ, BMT bone marrow transplantation, CGD chronic granulomatous disease, HSCT hematopoietic stem cell transplantation
Innate disorders Antibiotic prophylaxis Antifungal prophylaxis Cytokine replacement (IFNγ) for CGD Vaccinations (e.g., meningococcal) Ig replacement is sometimes indicated BMT, e.g., for CGD Gene therapy is a potential future treatment
  1. Ig immunoglobulin, IV intravenous, SC subcutaneous, CID combined immunodeficiency, SCID severe combined immunodeficiency, IFNγ interferon-γ, BMT bone marrow transplantation, CGD chronic granulomatous disease, HSCT hematopoietic stem cell transplantation