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A Patient With NEMO Deficiency, Disseminated M. szulgai, and Post-HSCT Inflammatory Disease
Ayelet Rosenthal1,2, Alka Goyal3, Sharon F Chen4
1Division of Pediatric Infectious Diseases, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA.
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.
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