15
Immune dysregulation
FHL No Yes No • Antimicrobials as needed
• Chemotherapy as needed
• Immunomodulators as needed
ALPS No Yes No
IPEX No Yes No
APECED No No No
Other Some Some No
Phagocytic cell defects
Neutropenia No Yes No • Avoid live bacterial vaccines: all
• Antimicrobial prophylaxis: all
• IFN-γ: CGD
• Surgical or dental debridement:
CGD, LAD-I
• Granulocyte transfusions:
CGD, LAD-I
• G-CSF: neutropenias
• Fucose: LAD-II
CGD No Yes Yes
LAD No Yes No
HIES type 1 Some Rare No
MSMD No Some No
Innate immune defects
NEMO deficiency,
other NF-κB defects
Yes Yes No • Avoid live vaccines: NF-κB
• PCP prophylaxis: NF-κB
• Antimicrobial prophylaxis:
NF-κB, CMCC
• G-CSF: WHIM syndrome
• Antiviral prophylaxis: HSE
CMCC No No No
WHIM syndrome Yes Some No
HSE No No No
EV No No No
Table 4. Summary of Therapeutic Considerations
for Primary Immunodeficiencies and Their
Complications (cont'd)
Diagnosis IgG
a
HSCT
Gene
therapy
