Successful Matched Related Bone Marrow Transplantation in a Patient with Autosomal Dominant Interferon Gamma Receptor 1 Deficiency.

ABSTRACT

This is a report of a successful bone marrow transplant in an IFN-γR1 patient with progressive mycobacterial infection. PURPOSE: Hematopoietic cell transplant in patients with interferon gamma receptor deficiencies has been fraught with challenges, not the least of which is failure of engraftment and infectious complications. METHODS: This is a report of a successful hematopoietic cell transplant in an actively infected patient of advanced age. RESULTS: This case report shows successful engraftment and resolution of infection posttransplant using a matched related donor in a single institution. CONCLUSION: A successful curative HCT despite persistent, disseminated, nontuberculous mycobacterial infection in a patient with AD-IFNγR1 suggests that this approach, while difficult, may be useful in other patients with otherwise refractory disease.