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WOS: 000536674800121 …

Amaç: Otoimmun HemolitikAnemiler (OİHA) eritrosit yüzey membranına bağlanan otoantikorlar sonucueritrositlerin erken parçalanması ile karakterizedirler. Evans Sendromu (ES), OİHAgeliştiği sırada veya anemi geliştikten sonra immün trombositopeni gelişen birhemolitik anemidir. Bu çalışmada OİHA ve ES tanısı ile izlenen çocuk hastalarındemografik, klinik ve laboratuvar özelliklerinin, klinik seyirlerinin, tedaviyecevaplarının, ve prognozlarına etki eden faktörlerin değerlendirilmesinamaçlandık.Yöntem: Haziran 2006- Haziran 2011 tarihleriarasında Ondokuzmayıs Üniversitesi Çocuk Hematoloji Bilim Dalı’nda takip edilenOİHA ve ES tanılı hastaların dosyaları geriyedönük olarak incelendi. Otoimmunhemolitik anemi tanısı, aynı zaman diliminde hemoglobin konsantrasyonu11g/dl’den düşük, direkt antiglobintesti (DAT) pozitif ve periferik yaymada hemoliz bulgusu varlığının üçünün debulunduğu hastalara konuldu. Talasemimajor hastaları, kemik iliği transplantasyonu (KİT) sonrası gelişen hemolitikanemiler ve yenidoğan döneminde görülen Rh ve ABO uygunsuzluğuna bağlıhemolitik anemiler çalışmaya dahil edilmedi. Bu hastaların demografik, klinik ve laboratuvar özellikleri, etiyolojiderol oynayan faktörleri, klinik seyirleri, tedaviye cevapları ve sağ kalımları kaydedildi. Bulgular: ÇalışmamızaOİHA ve ES tanısı olan yaş ortancası 36 (12-44) [ortanca (ÇAA)] ay olan, 21(%57’si erkek) hasta dahil edildi. Evans sendromlu 7 olgudan 4’ünde (%57) ‘çift negatif T lenfosit’ pozitifliğiolduğu için bu hastalar otoimmün lenfoproliferatif sendrom (OLPS) tanısı alarakçalışma dışı bırakıldı. Yirmi bir hastanın 18’i (%85) OİHA, 3’ü (%15) ES idi.OİHA’lı hastaların 13’ü (%72) primer OİHA 5’i (%28) sekonder OİHA idi. İzole OİHA18 hastamızın tedaviye cevap durumu incelendiğinde; 16’sının (%88) tamremisyona girip tedavisinin kesildiği, 1’inin (%6) halen tedavi almakta olduğu,1’inin ise (%6) eksitus olduğu tespit edildi.Tartışma: Tam remisyona girenhastalarımızın yaklaşık yarısı standart steroid tedavisi ile remisyona girememişve bu hastalara ilave diğer tedaviler verilmiştir. Otoimmun hemolitik anemiler ve ES çocuklukçağında nadir görülmesine rağmen mortalite ve morbiditeleri fazladır.

Amaç:Bu çalışmada; akut myeloblastik lösemi (AML) tanısı ile kemoterapi bloklarıalan çocuk hastalara verilmiş olan toplam eritrosit, random trombosit ve afereztrombosit süspansiyonun, hastanın o dönemde almış olduğu kemoterapi (KT) bloğuile olan ilişkisi değerlendirilmiştir.Gereçve Yöntemler: AML tanısıile takip edilen toplam 146 hastanın transfüzyon ihtiyacı, hastaların kanbankası verilerine girilerek tedavi süresince, torba sayısı ve tipiolarak eritrosit, random trombosit ve trombosit süspansiyonu olarak belirlendi.Bulgular:Toplam37 hastanın tanı konulduğu andan tedavinin bittiği döneme kadarki süreçte hastabaşı ortalama toplam eritrosit kullanımı 27 (10-102) adetti. KT bloklarıesnasında hem eritrosit hem de aferez trombosit kullanımının en fazla olduğublok Aie bloğu idi. Bu blokta ki ortalama kişi başı eritrosit kullanımı 8 (2-20) adet iken, aferez trombositkullanımı 8,5 (1-28) adet olarak saptanmıştır. Random trombosit kullanım adetiise kişi başı ortalama 20 (7-139) adet iken, en fazla random trombosit kullanılanKT bloğu olan İdame’de ki ortalama kişi başı random trombosit kullanım adeti 27(1-102) olarak saptanmıştır.Sonuç: AML’liçocuklarda KT süresince çok sayıda kan ve kan ürünü transfüzyonu gereklidir. Butransfüzyonun etkinliği akut löseminin tipine, hastanın yaşına, cinsiyetine,hastanın aldığı KT bloğu ile o dönem aldığı KT ilaçlarına ya da hastanın odönemde ki enfeksiyon, sepsis, yaygın tüketim koagulopatisi gibi klinik birtabloda olup olmaması gibi çok sayıda etkene bağlı olabilir., Aim: This study evaluates the association of totalerythrocyte, random platelet and apheresis platelet suspension given tochildren receiving chemotherapy (CT) blocks with a diagnosis of acutemyeloblastic leukemia (AML) with the chemotherapy (CT) block the patientreceived in that period. Material and Methods: The transfusion need of a total of 146 patientsfollowed with a diagnosis of AML were determined as erythrocyte, randomplatelet and platelet suspension as number and type in bags from the moment thepatients were diagnosed to the period the treatment ended by entering the bloodbank data base records. Results: The number of total per patient average erythrocyteuse of a total of 37 patients during the process from the moment they were diagnosed to the period thetreatment ended was 27 (100-102). Of the CT blocks, the block in which botherythrocyte and apheresis platelet use was the highest was Aie block. While theaverage per person erythrocyte use was 8(2-20) in this block, apheresis platelet use was found as 8,5 (1-28). While thenumber of average per person random platelet use was 20 (7-139), the number ofaverage per person random platelet use was as 27 (1-102) in the Maintenanceblock, which is the CT block in which the most random platelet was used. Conclusion: In children withAML, a great number of blood and blood product transfusions are neededduring CT. The efficiency of these transfusions can depend on a great number offactors such as the type of acute leukemia, patient age, patient gender, the CTblock the patient is receiving, the CT drugs the patient is receiving in thatperiod and whether the patient has a clinical picture such as infection,sepsis, intense consumption coagulopathy during that period.

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 30±17.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p=0.019). 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, Objective: This study aimed to define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey in terms of time of diagnosis, clinical characteristics, mutational studies, clinical course, and treatment strategies.Materials and Methods: Data including clinical and laboratory characteristics and treatment strategies of JMML patients were collected retrospectively from pediatric hematology-oncology centers in Turkey. Results: Sixty-five children with JMML diagnosed between 2002 and 2016 in 18 institutions throughout Turkey were enrolled in the study. the median age at diagnosis was 17 months (min-max: 2-117 months). Splenomegaly was present in 92% of patients at the time of diagnosis. the median white blood cell, monocyte, and platelet counts were 32.9x109/L, 5.4x109/L, and 58.3x109/L, respectively. Monosomy 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 30±17.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p=0.019). 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 are not satisfactory and it is necessary to put more effort into this issue in Turkey

WOS:000426572200004, PubMed ID: 28179213, 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.

WOS: 000426572200004, PubMed ID: 28179213, Objective: This study aimed to define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey in terms of time of diagnosis, clinical characteristics, mutational studies, clinical course, and treatment strategies. Materials and Methods: Data including clinical and laboratory characteristics and treatment strategies of JMML patients were collected retrospectively from pediatric hematology-oncology centers in Turkey. Results: Sixty-five children with JMML diagnosed between 2002 and 2016 in 18 institutions throughout Turkey were enrolled in the study. The median age at diagnosis was 17 months (min-max: 2-117 months). Splenomegaly was present in 92% of patients at the time of diagnosis. The median white blood cell, monocyte, and platelet counts were 32.9x10(9)/L, 5.4x10(9)/L, and 58.3x10(9)/L, respectively. Monosomy 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 30 +/- 17.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p=0.019). Older age at diagnosis (>2 years), platelet count of less than 40x10(9)/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 are not satisfactory and it is necessary to put more effort into this issue in Turkey.

Korkmaz, Muhammet Furkan/0000-0001-5440-7955 WOS: 000388276400007 …