Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients

Yıl: 2018 Cilt: 35 Sayı: 1 Sayfa Aralığı: 27 - 34 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022

Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients

Öz:
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
Anahtar Kelime:

Konular: Hematoloji

Türkiye’de Juvenil Miyelomonositik Lösemi: Altmış Beş Hastanın Retrospektif Analizi

Öz:
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
Anahtar Kelime:

Konular: Hematoloji
Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
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APA Tüfekçi Ö, Kocak U, Kaya Z, YENİCESU I, albayrak c, Albayrak D, BENGOA Ş, Patıroglu T, Karakukcu M, UNAL E, ince e, İLERİ D, ERTEM M, CELKAN T, ÖZDEMİR G, Sarper N, kaçar d, YARALI H, ozbek n, KÜPESİZ O, KARAPINAR T, Vergin C, ÇALISKAN Ü, Tokgöz H, EVİM M, BAYTAN B, meral gunes a, YILMAZ KARAPINAR D, KARAMAN S, UYGUN V, Karasu G, YEŞİLİPEK M, KOÇ A, ERDURAN E, Atabay B, Öniz H, ÖREN H (2018). Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. , 27 - 34.
Chicago Tüfekçi Özlem,Kocak Ulker,Kaya Zuhre,YENİCESU IDIL,albayrak canan,Albayrak Davut,BENGOA Şebnem Yılmaz,Patıroglu Türkan,Karakukcu Musa,UNAL Ekrem,ince elif,İLERİ Dilber Talia,ERTEM Mehmet,CELKAN TÜLIN TIRAJE,ÖZDEMİR Gül Nihal,Sarper Nazan,kaçar dilek,YARALI HÜSNİYE NEŞE,ozbek namik yasar,KÜPESİZ Osman Alphan,KARAPINAR Tuba,Vergin Canan,ÇALISKAN ÜMRAN,Tokgöz Hüseyin,EVİM Melike Sezgin,BAYTAN Birol,meral gunes adalet,YILMAZ KARAPINAR Deniz,KARAMAN SERAP,UYGUN Vedat Uygun,Karasu Gulsun,YEŞİLİPEK Mehmet Akif,KOÇ Ahmet,ERDURAN Erol,Atabay Berna,Öniz Haldun,ÖREN Hale Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. (2018): 27 - 34.
MLA Tüfekçi Özlem,Kocak Ulker,Kaya Zuhre,YENİCESU IDIL,albayrak canan,Albayrak Davut,BENGOA Şebnem Yılmaz,Patıroglu Türkan,Karakukcu Musa,UNAL Ekrem,ince elif,İLERİ Dilber Talia,ERTEM Mehmet,CELKAN TÜLIN TIRAJE,ÖZDEMİR Gül Nihal,Sarper Nazan,kaçar dilek,YARALI HÜSNİYE NEŞE,ozbek namik yasar,KÜPESİZ Osman Alphan,KARAPINAR Tuba,Vergin Canan,ÇALISKAN ÜMRAN,Tokgöz Hüseyin,EVİM Melike Sezgin,BAYTAN Birol,meral gunes adalet,YILMAZ KARAPINAR Deniz,KARAMAN SERAP,UYGUN Vedat Uygun,Karasu Gulsun,YEŞİLİPEK Mehmet Akif,KOÇ Ahmet,ERDURAN Erol,Atabay Berna,Öniz Haldun,ÖREN Hale Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. , 2018, ss.27 - 34.
AMA Tüfekçi Ö,Kocak U,Kaya Z,YENİCESU I,albayrak c,Albayrak D,BENGOA Ş,Patıroglu T,Karakukcu M,UNAL E,ince e,İLERİ D,ERTEM M,CELKAN T,ÖZDEMİR G,Sarper N,kaçar d,YARALI H,ozbek n,KÜPESİZ O,KARAPINAR T,Vergin C,ÇALISKAN Ü,Tokgöz H,EVİM M,BAYTAN B,meral gunes a,YILMAZ KARAPINAR D,KARAMAN S,UYGUN V,Karasu G,YEŞİLİPEK M,KOÇ A,ERDURAN E,Atabay B,Öniz H,ÖREN H Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. . 2018; 27 - 34.
Vancouver Tüfekçi Ö,Kocak U,Kaya Z,YENİCESU I,albayrak c,Albayrak D,BENGOA Ş,Patıroglu T,Karakukcu M,UNAL E,ince e,İLERİ D,ERTEM M,CELKAN T,ÖZDEMİR G,Sarper N,kaçar d,YARALI H,ozbek n,KÜPESİZ O,KARAPINAR T,Vergin C,ÇALISKAN Ü,Tokgöz H,EVİM M,BAYTAN B,meral gunes a,YILMAZ KARAPINAR D,KARAMAN S,UYGUN V,Karasu G,YEŞİLİPEK M,KOÇ A,ERDURAN E,Atabay B,Öniz H,ÖREN H Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. . 2018; 27 - 34.
IEEE Tüfekçi Ö,Kocak U,Kaya Z,YENİCESU I,albayrak c,Albayrak D,BENGOA Ş,Patıroglu T,Karakukcu M,UNAL E,ince e,İLERİ D,ERTEM M,CELKAN T,ÖZDEMİR G,Sarper N,kaçar d,YARALI H,ozbek n,KÜPESİZ O,KARAPINAR T,Vergin C,ÇALISKAN Ü,Tokgöz H,EVİM M,BAYTAN B,meral gunes a,YILMAZ KARAPINAR D,KARAMAN S,UYGUN V,Karasu G,YEŞİLİPEK M,KOÇ A,ERDURAN E,Atabay B,Öniz H,ÖREN H "Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients." , ss.27 - 34, 2018.
ISNAD Tüfekçi, Özlem vd. "Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients". (2018), 27-34.
APA Tüfekçi Ö, Kocak U, Kaya Z, YENİCESU I, albayrak c, Albayrak D, BENGOA Ş, Patıroglu T, Karakukcu M, UNAL E, ince e, İLERİ D, ERTEM M, CELKAN T, ÖZDEMİR G, Sarper N, kaçar d, YARALI H, ozbek n, KÜPESİZ O, KARAPINAR T, Vergin C, ÇALISKAN Ü, Tokgöz H, EVİM M, BAYTAN B, meral gunes a, YILMAZ KARAPINAR D, KARAMAN S, UYGUN V, Karasu G, YEŞİLİPEK M, KOÇ A, ERDURAN E, Atabay B, Öniz H, ÖREN H (2018). Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. Turkish Journal of Hematology, 35(1), 27 - 34.
Chicago Tüfekçi Özlem,Kocak Ulker,Kaya Zuhre,YENİCESU IDIL,albayrak canan,Albayrak Davut,BENGOA Şebnem Yılmaz,Patıroglu Türkan,Karakukcu Musa,UNAL Ekrem,ince elif,İLERİ Dilber Talia,ERTEM Mehmet,CELKAN TÜLIN TIRAJE,ÖZDEMİR Gül Nihal,Sarper Nazan,kaçar dilek,YARALI HÜSNİYE NEŞE,ozbek namik yasar,KÜPESİZ Osman Alphan,KARAPINAR Tuba,Vergin Canan,ÇALISKAN ÜMRAN,Tokgöz Hüseyin,EVİM Melike Sezgin,BAYTAN Birol,meral gunes adalet,YILMAZ KARAPINAR Deniz,KARAMAN SERAP,UYGUN Vedat Uygun,Karasu Gulsun,YEŞİLİPEK Mehmet Akif,KOÇ Ahmet,ERDURAN Erol,Atabay Berna,Öniz Haldun,ÖREN Hale Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. Turkish Journal of Hematology 35, no.1 (2018): 27 - 34.
MLA Tüfekçi Özlem,Kocak Ulker,Kaya Zuhre,YENİCESU IDIL,albayrak canan,Albayrak Davut,BENGOA Şebnem Yılmaz,Patıroglu Türkan,Karakukcu Musa,UNAL Ekrem,ince elif,İLERİ Dilber Talia,ERTEM Mehmet,CELKAN TÜLIN TIRAJE,ÖZDEMİR Gül Nihal,Sarper Nazan,kaçar dilek,YARALI HÜSNİYE NEŞE,ozbek namik yasar,KÜPESİZ Osman Alphan,KARAPINAR Tuba,Vergin Canan,ÇALISKAN ÜMRAN,Tokgöz Hüseyin,EVİM Melike Sezgin,BAYTAN Birol,meral gunes adalet,YILMAZ KARAPINAR Deniz,KARAMAN SERAP,UYGUN Vedat Uygun,Karasu Gulsun,YEŞİLİPEK Mehmet Akif,KOÇ Ahmet,ERDURAN Erol,Atabay Berna,Öniz Haldun,ÖREN Hale Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. Turkish Journal of Hematology, vol.35, no.1, 2018, ss.27 - 34.
AMA Tüfekçi Ö,Kocak U,Kaya Z,YENİCESU I,albayrak c,Albayrak D,BENGOA Ş,Patıroglu T,Karakukcu M,UNAL E,ince e,İLERİ D,ERTEM M,CELKAN T,ÖZDEMİR G,Sarper N,kaçar d,YARALI H,ozbek n,KÜPESİZ O,KARAPINAR T,Vergin C,ÇALISKAN Ü,Tokgöz H,EVİM M,BAYTAN B,meral gunes a,YILMAZ KARAPINAR D,KARAMAN S,UYGUN V,Karasu G,YEŞİLİPEK M,KOÇ A,ERDURAN E,Atabay B,Öniz H,ÖREN H Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. Turkish Journal of Hematology. 2018; 35(1): 27 - 34.
Vancouver Tüfekçi Ö,Kocak U,Kaya Z,YENİCESU I,albayrak c,Albayrak D,BENGOA Ş,Patıroglu T,Karakukcu M,UNAL E,ince e,İLERİ D,ERTEM M,CELKAN T,ÖZDEMİR G,Sarper N,kaçar d,YARALI H,ozbek n,KÜPESİZ O,KARAPINAR T,Vergin C,ÇALISKAN Ü,Tokgöz H,EVİM M,BAYTAN B,meral gunes a,YILMAZ KARAPINAR D,KARAMAN S,UYGUN V,Karasu G,YEŞİLİPEK M,KOÇ A,ERDURAN E,Atabay B,Öniz H,ÖREN H Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. Turkish Journal of Hematology. 2018; 35(1): 27 - 34.
IEEE Tüfekçi Ö,Kocak U,Kaya Z,YENİCESU I,albayrak c,Albayrak D,BENGOA Ş,Patıroglu T,Karakukcu M,UNAL E,ince e,İLERİ D,ERTEM M,CELKAN T,ÖZDEMİR G,Sarper N,kaçar d,YARALI H,ozbek n,KÜPESİZ O,KARAPINAR T,Vergin C,ÇALISKAN Ü,Tokgöz H,EVİM M,BAYTAN B,meral gunes a,YILMAZ KARAPINAR D,KARAMAN S,UYGUN V,Karasu G,YEŞİLİPEK M,KOÇ A,ERDURAN E,Atabay B,Öniz H,ÖREN H "Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients." Turkish Journal of Hematology, 35, ss.27 - 34, 2018.
ISNAD Tüfekçi, Özlem vd. "Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients". Turkish Journal of Hematology 35/1 (2018), 27-34.