Long-term follow-up and prognosis of XXXX in Yugoslavia: is there a role for early bone marrow transplantation?

@entity1 @entity5572 @entity295 @entity3136 @entity799 @entity55 @entity281 @entity2381 @entity1030 @entity1117 @entity3298
We report the long-term follow-up of 12 pediatric-aged @entity1 with @entity1117 ( @entity1117 ). The mean age at the onset of @entity281 was 5 months with a median delay in diagnosis of 2.5 years. Bacille Calmette-Gu  rin @entity3298 was the most common presenting @entity281 (6) followed by suppurative @entity3298 (4), @entity5572 (1), or @entity295 (1). Prophylaxis with @entity799 was recommended to all @entity1 . During the mean follow-up of 10 years (range, 4-23 years) @entity55 was the most prevalent @entity281 (91%) followed by @entity3298 (83%), @entity3136 (58%), and @entity5572 (25%). Seven (58%) @entity1 developed @entity1030 due to grossly delayed diagnosis (3) or poor compliance with antimicrobial prophylaxis (4). Five (41%) @entity1 died during the second decade of life of @entity2381 (3) or @entity1030 (2). Probability of survival into the third decade of life was estimated to be only 19%. We argue that HLA-identical bone marrow transplantation (BMT), if possible, should be attempted at early age because of significant morbidity and mortality in adolescence. BMT also should be considered in @entity1 who suffer severe @entity281 despite antimicrobial prophylaxis or @entity1 with evidence of @entity1030 . Possibility of elective BMT from unrelated donors remains to be carefully evaluated.
