Table 3 Strategies for the treatment and management of PIDs.
From: Primary immunodeficiency
Supportive | Definitive | |
|---|---|---|
CIDs/SCID | â–º Ig replacement therapy (IV or SC) â–º Antibiotic prophylaxis â–º Antifungal prophylaxis â–º Aggressive management of established infections â–º Infectious precautions when hospitalized â–º Withhold all live vaccines | â–º BMT â–º HSCT â–º Gene therapy a possibility for some SCIDs |
B-cell disorders | â–º Ig replacement therapy (IV or SC) â–º Antibiotic prophylaxis â–º Antifungal prophylaxis depending upon etiology â–º Hearing assessment â–º Assessment of pulmonary status and function â–º Close monitoring for co-morbidities | â–º Gene therapy is a potential future treatment in some patients |
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 |