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Severe Combined ImmunoDeficiency
Answer:SCID.
Patients with SCID are unable to produce functional antibodies.
Although the basic defect in SCID is T-cell dysfunction, B cells are unable to produce functional antibody due to lack of T-cell help.
Therefore, routine IVIg replacement therapy is indicated until more definitive therapy (eg, bone marrow transplantation) can be performed.
Isolated IgG2 subclass deficiency without evidence of functional antibody production deficiency is not an indication for IVIg therapy.
X-linked SCID is the 1st and only disease in which stem cell therapy is successful and fda approved.
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