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Long-term follow-up and prognosis of chronic granulomatous disease in Yugoslavia: Is there a role for early bone marrow transplantation?

Lookup NU author(s): Dr Desa Lilic, Dr Mario Abinun


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We report the long-term follow-up of 12 pediatric-aged patients with chronic granulomatous disease (CGD). The mean age at the onset of infections was 5 months with a median delay in diagnosis of 2.5 years. Bacille Calmette-Guérin lymphadenitis was the most common presenting infection (6) followed by suppurative lymphadenitis (4), liver abscess (1), or Salmonella sepsis (1). Prophylaxis with cotrimoxazole was recommended to all patients. During the mean follow-up of 10 years (range, 4-23 years) pneumonitis was the most prevalent infection (91%) followed by lymphadenitis (83%), aphtous stomatitis (58%), and liver abscesses (25%). Seven (58%) patients developed chronic lung disease due to grossly delayed diagnosis (3) or poor compliance with antimicrobial prophylaxis (4). Five (41%) patients died during the second decade of life of aspergillosis (3) or chronic lung disease (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 patients who suffer severe infections despite antimicrobial prophylaxis or patients with evidence of chronic lung disease. Possibility of elective BMT from unrelated donors remains to be carefully evaluated.

Publication metadata

Author(s): Pasic S, Minic A, Minic P, Veljkovic D, Lilic D, Slavkovic B, Pejnovic N, Abinun M

Publication type: Article

Publication status: Published

Journal: Journal of Clinical Immunology

Year: 2003

Volume: 23

Issue: 1

Pages: 55-61

Print publication date: 01/01/2003

ISSN (print): 0271-9142

ISSN (electronic): 1573-2592

Publisher: Springer


DOI: 10.1023/A:1021952315651

PubMed id: 12645860


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