18
Combined B- and T-Cell Immunodeficiencies
Severe combined immunodeficiency (SCID)
Î SS 26. SCID should be considered in the differential diagnosis when
an infant presents with recurrent, persistent, or severe bacterial, viral,
or fungal infections or failure to thrive. (C)
Î SS 27. Patients with SCID or suspected SCID should receive IgG
replacement therapy. (C)
Î SS 28. Patients with SCID or suspected SCID should be protected from
exposure to infectious agents. (C)
Î SS 29. Patients with SCID should receive PCP prophylaxis. (C)
Î SS 30. Early signs of infection should be promptly investigated and
antimicrobial regimens initiated and for prolonged periods. (C)
Î SS 31. Polyethylene glycol (PEG)-conjugated ADA (PEG-ADA) should
be administered to patients with SCID caused by ADA deficiency if
HSCT or gene therapy is unavailable. (C)
Î SS 32. A suspicion of SCID should be considered an urgent clinical
condition. (C)
Î SS 33. Patients with SCID should be immunologically reconstituted by
means of HSCT or gene therapy. (B)
Combined B- and T-Cell Immunodeficiencies
