A Patient With NEMO Deficiency, Disseminated M. szulgai, and Post-HSCT Inflammatory Disease.

BACKGROUND

Disseminated mycobacterium poses a significant risk for patients with NEMO deficiency. Hematopoietic stem cell transplant (HSCT) corrects the NEMO defect in hematopoietic cells thus treating the immunodeficiency.

METHODS

We present a patient with NEMO deficiency who successfully underwent HSCT despite a disseminated Mycobacterium szulgai infection.

RESULTS

Despite successful engraftment and resolution of the mycobacterium infection, he developed inflammatory disease leading to his death.

CONCLUSION

HSCT does not cure all aspects of the NEMO protein defect and posttransplant inflammatory diseases involving nonhematopoietic cell types may manifest clinically.