A Case Diagnosed with Chronic Granulomatous Disease After Disseminated Infection Following BCG Vaccination

BCG (Bacillus Calmette-Guérin) aşısı, çocukları miliyer tüberküloz (TB) ve TB menenjitinden korumak amacıyla Dünya Sağlık Örgütü'nün önerisiyle yaygın olarak kullanılan bir aşıdır. Aşıya bağlı ciddi yan etkiler, sıklıkla altta yatan immün yetmezlik varlığında görülür. Bu raporda, kronik granülomatöz hastalık (KGH) taşıyıcısı olduğu bilinmeden BCG aşısı uygulanan ve aşı sonrası disemine BCG enfeksiyonu gelişen bir bebek olgu sunulmuştur. Bilinen bir sağlık problemi olmayan 3 ay 28 günlük bir kız çocuğunda, BCG aşısı sonrası yüksek ateş, sol aksiller lenfadenopati ve hepatosplenomegali gelişmiştir. Olgunun aksiller lenf bezi eksizyon materyalinin Löwenstein-Jensen besiyerinde yapılan kültüründen aside dirençli basil (ARB) izole edilmiş ve Mycobacterium bovis olarak tanımlanmıştır. Lenf bezi dokusu örneğinden yapılan polimeraz zincir reaksiyonu testinde Mycobacterium tuberculosis kompleks DNA'sı pozitif bulunmuştur. Hastaya rifampisin 20 mg/kg + izoniyazid 10 mg/kg + etambutol 15 mg/kg + streptomisin 30 mg/kg dozunda antitüberküloz tedavi başlanmıştır. İmmün yetmezlik açısından değerlendirilmek üzere dihidroamin ve LAD (lenfosit adezyon defekti) testleri çalışılmış ve alınan sonuçlara göre hastaya otozomal resesif tipte KGH tanısı konulmuştur. Olgunun yenidoğan döneminde tespit edilen ya da ailesinde bilinen herhangi bir immün yetmezlik öyküsü olmadığı öğrenilmiştir. Hastaya, tüberküloz için endemik bölgelerde yaşayan KGH hastalarında kullanımı önerilen interferon-gama tedavisi uygulanmıştır. Antitüberküloz tedavinin 15. gününde olgunun ateşi düşmüş, 35. gün tedavisine evde devam etmek üzere taburcu edilmiştir. Poliklinikten takibine devam edilen hastanın ek bir şikayeti olmamış ve üçüncü ayda hepatosplenomegalisi tamamen düzelmiştir. Sonuç olarak, KGH taşıyıcılarında BCG aşısı kontrendike olduğundan, ailesinde KGH öyküsü olan yenidoğanlar immünolojik olarak tetkik edilmeli ve sonuçlar elde edilinceye kadar BCG aşısından kaçınılmalıdır. Aile öyküsü olmayan ve BCG aşısı sonrası mikobakteri enfeksiyonu tanısı konulan bebekler mutlaka immün yetmezlik açısından değerlendirilmelidirBCG (Bacillus Calmette-Guérin) vaccine is a widely used vaccine with the recommendation of World Health Organization to protect children against miliary tuberculosis (TB) and TB meningitis. Severe side effects related to this vaccine mostly manifest in the presence of underlying immunosuppressive disease. In this report, an infant case with unknown chronic granulomatous disease (CGD) who developed disseminated BCG infection after administration of BCG vaccine, was presented. High fever, left axillary lymphadenopathy and hepatosplenomegaly have developed in a 3-month 28-day female infant, without a known health problem, following BCG vaccination. The acid-fast bacilli (ARB) was isolated from the material of excised lymph node cultivated in Löwenstein-Jensen medium, and the isolate was identifi ed as Mycobacterium bovis. Mycobacterium tuberculosis complex DNA was detected in the axillary lymph node sample by polymerase chain reaction. Anti-tuberculous treatment included 20 mg/kg of rifampicin + 10 mg/kg of isoniazid + 15 mg/kg of ethambutol + 30 mg/kg of streptomycin was started. The patient was then further evaluated for immunodefi ciency and on the basis of the results of dihydroamine and LAD (lymphocyte adhesion defect) tests, diagnosed as autosomal recessive CGD. Based on the anamnesis, there was no known immunodefi ciency history both in the case during neonatal period and her family members. Interferon-gamma therapy, which is recommended for the patients with CGD living in endemic areas, was initiated. Our patient's fever dropped at the 15th day of anti-tuberculosis treatment, and she was discharged on the 35th day and continued to receive treatment at home. The patient was followed up at outpatient clinic and had no additional complaints; her hepatosplenomegaly was back to normal at the third month. As a result, since BCG vaccine is contraindicated in CGD carriers, newborns with a family history of CGD should be immunologically examined and BCG vaccine should be avoided until the results are obtained. In addition, newborns without a family history, diagnosed as disseminated mycobacterial infection following BCG vaccination, should be evaluated for an underlying immunodefi ciency conditio

Antifungal Prophylaxis and Treatment of Breakthrough Invasive Fungal Diseases in High-Risk Hematology Patients: A Prospective Observational Multicenter Study

We aimed to investigate the approaches for antifungal prophylaxis (AFP) and antifungal treatment in breakthrough invasive fungal diseases (IFDs) under AFP in high-risk hematology patients. Patients ≥ 18-years who received chemotherapy for acute myeloid leukemia (AML), acute lymphoblastic leukemia (ALL) or a conditioning regimen for allogeneic hematopoietic stem cell transplantation (AHSCT) with a duration of neutropenia (< 500 cells/mm3) ≥ 10 days were included in a prospective multicenter observational study. Patients were followed until one week after recovery from neutropenia, discharge from the hospital, or death, which comes first to define the success of AFP. A total of 230 patients were recruited from 18 centers in seven months. Posaconazole prophylaxis was used in 134 (44 of whom failed) and 96 patients received fluconazole (28 of whom failed). The survival rate at 12 weeks after the initiation of AFP was higher in patients with successful prophylaxis (96.2% vs 56.9%, p < 0.001). IFDs were diagnosed in 27 patients. Duration of neutropenia was the only risk factor (OR: 1.03; 95% CI: 1.004–1.053) for development of IFDs. The types of breakthrough IFDs were; possible IFD in 15 patients, probable invasive aspergillosis (IA) in 9 patients, proven IA in 2 patients; and proven mucormycosis in 1 patient. Voriconazole was the drug of choice in 16 patients (5 of whom failed). Liposomal amphotericin B was used in the treatment of 8 patients (4 of whom failed). Posaconazole was the most frequently prescribed AFP in AML patients with high compliance to international guidelines. Approximately, one-third of ALL patients and AHSCT recipients received off-label posaconazole prophylaxis