Abstract • 155
Severe combined immunodeficiencies are a group of genetic disorders characterized by profoundly defective T lymphocytes (with or without B lymphocyte) differentiation that lead to early death without hematopoietic stem cell transplantation. Case M.T: 6 month old girl admitted to our clinic with complaints of persistent thrush, diarrhea, and coughing. She was the fourth child of consanguineous parents; a sister had died at 8 months of age. The patient had received BSG vaccination in her left deltoid muscle during her early neonatal period. Her symptoms began in the first month of life. She was diagnosed as T - B+ SCID and received a fully matched sibling marrow (EBMT protocol 1997) only 4 days after diagnosis. Engraftment followed by a Grade Ia GvhD was achieved on day 13. Persistent fever not explained by viral of fungal infections occured during follow-up. Shortly thereafter, disseminated Tbc spina ventosa was diagnosed by EZN positive smears, PCR, and culture (Myc Tbc complex). She was put on antituberculous chemotherapy with 4 culture susceptible agents (INH, RIF, Strep, Clofezamine) for 2 months followed by INH + RIF for another 4 months. She responded well with full immune reconstitution and both clinical and radiographic regression of Tbc infection. However, her BCG disease relapsed on the 6th month of anti-tbc chemotherapy and she was put on alternative agents (Amicacin + Ciprofloxamine) in addition to two major agents (INH + RIF).
