Correct!
4. 1 and 3 or 5. All of the above
Our patient was tested for T cells, NK cells and B cells (Figure 4).
Figure 4. Patient’s immune cell panel.
In SCID the body produces very few T cells and NK cells. B cells become defective in the absence of T cells. Most patients with X-SCID died before the age of 2 without treatment until universal screening identified those patients. Treating the patient with IgG allows the patients to usually live until adulthood. However, this patient also has mediastinal lymphadenopathy and a restrictive pulmonary function defect and has likely developed granulomatous and lymphocytic ILD (GLILD) as a complication of their X-SCID (3).
How is GLILD best treated? (Click on the correct answer to be directed to the sixth and final page)