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    Central Nervous System Fungal Infections in Children With Leukemia and Undergoing Hematopoietic Stem Cell Transplantation: A Retrospective Multicenter Study
    (Lippincott Williams & Wilkins, 2022) Karaman, Serap; Kebudi, Rejin; Kizilocak, Hande; Karakas, Zeynep; Demirag, Bengu; Evim, Melike S.; Yarali, Nese
    Background: Central nervous system fungal infections (CNSFI) are seen in patients with hematologic malignancies and have high morbidity and mortality. Because of their rarity, there is limited data on CNSFI in children with no established treatment protocols or guidelines. Materials and Methods: In this multicenter retrospective study. 51 pediatric patients with leukemia. 6 of whom had undergone bone marrow transplantation, with proven or probable CNSFI were evaluated. Fungal infections were defined as proven or probable based on European Organisation for Research and Treatment of Cancer criteria. Proven CNSFI was diagnosed by appropriate central nervous system (CNS) imaging or tissue sample findings in combi-nation with positive microbiological results of cerebrospinal fluid. A positive culture, microscopic evidence of hyphae, a positive result of the galactomannan assays are defined as positive microbiological evidence. Probable CNSFI was defined as appropriate CNS imaging findings together with proven or probable invasive fungal infections at another focus without CNS when there is no other explanatory condition. Data was collected by using the questionnaire form (Supplemental Digital Content 1, http://links lww.com/JPHO/A541). Results: Seventeen patients had proven, 34 patients had probable CNSFI. Headaches and seizures were the most common clinical findings. The median time between the onset of fever and diagnosis was 5 days. The most common fungal agent identified was Aspergillus. Sixteen patients received single-agent. 35 received combination antifungal therapy. Surgery was performed in 23 patients. Twenty-two patients (43%) died. 29 of the CNSFI episodes recovered with a 20% neurological sequelae. Conclusion: CNSFIs should be considered in the differential diagnosis in patients with leukemia and refractory/recurrent fever, headache, neurologicalocular symptoms, and a radiologic-serological evaluation should be performed immediately. Early diagnosis and prompt management, both medical and surgical, are essential for improving clinical outcomes.
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    Central Nervous System Fungal Infections in Children with Leukemia, Risk Factors and Outcome: A Multicentric Study
    (Amer Soc Hematology, 2018) Kebudi, Rejin; Karaman, Serap; Kizilocak, Hande; Karakas, Zeynep; Evim, Melike Sezgin; Demirag, Bengu; Kaya, Zuhre
    [Abstract Not Availabe]
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    Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients
    (2018) Tüfekçi, Özlem; Koçak, Ülker; Kaya, Zühre; Yenicesu, İdil; Albayrak, Canan; Albayrak, Davut; Tokgöz, Hüseyin; Çalışkan, Ümran; Yılmaz Bengoa, Şebnem; Patıroğlu, Türkan; Karakükçü, Musa; Ünal, Ekrem; Ünal İnce, Elif; İleri, Talia; Ertem, Mehmet; Celkan, Tiraje; Özdemir, Gül Nihal; Sarper, Nazan; Kaçar, Dilek; Yaralı, Neşe; Özbek, Namık Yaşar; Küpesiz, Alphan; Karapınar, Tuba; Vergin, Canan; Tokgöz, Hüseyin; Sezgin Evim, Melike; Baytan, Birol; Güneş, Adalet Meral; Yılmaz Karapınar, Deniz; Karaman, Serap; Uygun, Vedat; Karasu, Gülsun; Yeşilipek, Mehmet Akif; Koç, Ahmet; Erduran, Erol; Atabay, Berna; Öniz, Haldun; Ören, Hale
    Amaç: Türkiye'deki juvenil miyelomonositik lösemi (JMML) hastalarının durumunu, tanı zamanı, klinik özellikler, mutasyon çalışmaları, klinik gidiş ve tedavi stratejileri açısından ortaya koymaktır.Gereç ve Yöntemler: Ülkemizdeki pediatrik hematoloji ve onkoloji kliniklerinden veri istenerek, JMML tanısı ile takip ve tedavisi yapılan hastaların klinik ve laboratuvar bulguları geriye dönük olarak değerlendirildi.Bulgular: On sekiz merkezden, 2002-2016 tarihleri arasında JMML tanısı alan toplam 65 hasta çalışmaya dahil edildi. Ortanca tanı yaşı 17 ay idi (2-117 ay). Splenomegali tanıda %92 hastada vardı. Ortanca lökosit, monosit ve trombosit sayıları sırasıyla 32,9x109/L, 5,4x109/L ve 58,3x109/L idi. Monozomi 7, %18 hastada saptanmıştı. JMML mutasyonları 32 hastada (%49) çalışılmış olup, en sık rastlanan mutasyon PTPN11 idi. Hematopoetik kök hücre nakli (HKHN) 7 was present in 18% of patients. JMML mutational analysis was performed in 32 of 65 patients (49%) and PTPN11 was the most common mutation. Hematopoietic stem cell transplantation (HSCT) could only be performed in 28 patients (44%), the majority being after the year 2012. The most frequent reason for not performing HSCT was the inability to find a suitable donor. The median time from diagnosis to HSCT was 9 months (min-max: 2-63 months). The 5-year cumulative survival rate was 33% and median estimated survival time was 3017.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p0.019). Older age at diagnosis (2 years), platelet count of less than 40x109/L, and PTPN11 mutation were the factors significantly associated with shorter survival time. Conclusion: Although there has recently been improvement in terms of definitive diagnosis and HSCT in JMML patients, the overall results.