14
Treatment
Table 4. Summary of Therapeutic Considerations
for Primary Immunodeficiencies and Their
Complications
Diagnosis IgG
a
HSCT
Gene
therapy
CIDs
SCID (IL2RG, ADA) Yes Yes Yes • Avoid live vaccines: all
• PCP prophylaxis: all SCID,
CD40, CD40L
• Antimicrobials as needed
• Blood products irradiated,
CMV
–
: all
• ADA: PEG-ADA
• CD40, CD40L: G-CSF
SCID (other) Yes Yes No
CD40L deficiency Yes Yes No
Other CID Yes Many No
Immunodeficiency syndromes
WAS Yes Yes Yes • Avoid live vaccines: many
• Multidisciplinary care: many
• WAS: splenectomy
• DGS: thymus transplantation
• Immunomodulation as needed
• Chemotherapy as needed
AT Some No No
DGS Some No No
Other syndromes Some Some No
Antibody deficiency
Agammaglobulinemia Yes No No • Avoid live vaccines:
agammaglobulinemia, CVID
• Antibiotics: all
• Splenectomy: CVID
• Immunomodulation: CVID
• Chemotherapy: CVID
• Pneumococcal vaccine:
SIGAD, IGGSD, SAD
CVID Yes Rare No
Other antibody
deficiency
Yes No No
Treatment
