2008 Volume 22 Issue 2 Pages 129-132
Omenn Syndrome is a form of severe congenital combined immunodeficiency characterized by erythrodermia, hepatosplenomegaly, lymphadenopathy, and alopecia. Most patients with Omenn Syndrome are threatened due to severe infections; thus, they are generally treated with cord blood or bone marrow stem cell transplantation in early childhood. We here report a patient with Omenn Syndrome who suffered Mycobacterium avium complex (MAC) infection in the neck and axillary lymph nodes after cord blood stem cell transplantation. Thispatient was mainly treated with ethambutol, rifampicin, clarithromycin, and azithromycin, and the repeated incisions and drainages of lymph node lesions were performed for 2 years after the onset of MAC infection. These results suggested that administration of antituberculosis drugs and macrolide antibiotics, and surgical treatment are effective for systemic MAC infections after hematopoietic cell transplantation.
