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

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Turkish Journal of Hematology

Türkiye’de Juvenil Miyelomonositik Lösemi: Altmış Beş Hastanın Retrospektif Analizi [Turk J Hematol]

Turk J Hematol. 2018; 35(1): 27-34 | DOI: 10.4274/tjh.2017.0021

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

Özlem Tüfekçi¹, Ülker Koçak², Zühre Kaya², İdil Yenicesu², Canan Albayrak³, Davut Albayrak³, Şebnem Yılmaz Bengoa¹, Türkan Patıroğlu⁴, Musa Karakükçü⁴, Ekrem Ünal⁴, Elif Ünal İnce⁵, Tali İleri⁵, Mehmet Ertem⁵, Tiraje Celkan⁶, Gül Nihal Özdemir⁶, Nazan Sarper⁷, Dilek Kaçar⁸, Neşe Yaralı⁸, Namık Yaşar Özbek⁸, Alphan Küpesiz⁹, Tuba Karapınar¹⁰, Canan Vergin¹⁰, Ümran Çalışkan¹¹, Hüseyin Tokgöz¹¹, Melike Sezgin Evim¹², Birol Baytan¹², Adalet Meral Güneş¹², Deniz Yılmaz Karapınar¹³, Serap Karaman¹⁴, Vedat Uygun¹⁵, Gülsun Karasu¹⁵, Mehmet Akif Yeşilipek¹⁵, Ahmet Koç¹⁶, Erol Erduran¹⁷, Berna Atabay¹⁸, Haldun Öniz¹⁸, Hale Ören¹
¹Dokuz Eylül University Faculty of Medicine, Department of Pediatric Hematology, İzmir, Turkey
²Gazi University Faculty of Medicine, Department of Pediatric Hematology, Ankara, Turkey
³Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology, Samsun, Turkey
⁴Erciyes University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Kayseri, Turkey
⁵Ankara University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Ankara, Turkey
⁶İstanbul University Cerrahpaşa Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey
⁷Kocaeli University Faculty of Medicine, Department of Pediatric Hematology, Kocaeli, Turkey
⁸Ankara Children’s Hematology and Oncology Training and Research Hospital, Ankara, Turkey
⁹Akdeniz University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Antalya, Turkey
¹⁰Dr. Behçet Uz Children Training and Research Hospital, Clinic of Pediatric Hematology and Oncology, İzmir, Turkey
¹¹Necmettin Erbakan University Meram Faculty of Medicine, Department of Pediatric Hematology, Konya, Turkey
¹²Uludağ University Faculty of Medicine, Department of Pediatric Hematology, Bursa, Turkey
¹³Ege University Faculty of Medicine, Department of Pediatric Hematology, İzmir, Turkey
¹⁴Şişli Hamidiye Etfal Training and Research Hospital, Clinic of Pediatric Hematology and Oncology, İstanbul, Turkey
¹⁵Bahçeşehir University Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey
¹⁶Marmara University Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey
¹⁷Karadeniz Technical University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Trabzon, Turkey
¹⁸Tepecik Training and Research Hospital, Clinic of Pediatric Hematology and Oncology, İzmir, Turkey

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 Yöntem: 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) hastaların ancak %44’üne uygulanabilmiş olup, nakillerin büyük bir oranı 2012 yılından sonra yapılmıştı. Nakil yapılamamasının en sık nedeni uygun donör bulunamamasıydı. Tanı aldıktan nakile kadar geçen ortalama süre 9 ay (2-63 ay) olarak saptandı. Tüm hastalarda 5 yıllık kümülatif sağkalım oranı %33, ortanca tahmini yaşam süresi ise 30±17,4 ay (%95 CI: 0-64,1) olarak bulundu. Sağkalım süresi HKHN yapılan hastalarda anlamlı olarak daha uzundu (log-rank p=0,019). Tanıda 2 yaşın üstünde olmak, trombosit sayısının 40x109/L’nin altında saptanması ve PTPN11 mutasyon varlığı yaşam süresini anlamlı olarak kısaltan faktörler olarak bulundu.
Sonuç: Ülkemizde her ne kadar son dönemlerde JMML hastalarında kesin tanı ve HKHN açısından iyileşme kaydedilmiş olsa da genel sonuç tatminkar değildir ve bu konu ile ilgili daha fazla çaba göstermeye gerek vardır.

Anahtar Kelimeler: Hematopoetik kök hücre nakli, Juvenil miyelomonositik lösemi, Türkiye

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

Özlem Tüfekçi¹, Ülker Koçak², Zühre Kaya², İdil Yenicesu², Canan Albayrak³, Davut Albayrak³, Şebnem Yılmaz Bengoa¹, Türkan Patıroğlu⁴, Musa Karakükçü⁴, Ekrem Ünal⁴, Elif Ünal İnce⁵, Tali İleri⁵, Mehmet Ertem⁵, Tiraje Celkan⁶, Gül Nihal Özdemir⁶, Nazan Sarper⁷, Dilek Kaçar⁸, Neşe Yaralı⁸, Namık Yaşar Özbek⁸, Alphan Küpesiz⁹, Tuba Karapınar¹⁰, Canan Vergin¹⁰, Ümran Çalışkan¹¹, Hüseyin Tokgöz¹¹, Melike Sezgin Evim¹², Birol Baytan¹², Adalet Meral Güneş¹², Deniz Yılmaz Karapınar¹³, Serap Karaman¹⁴, Vedat Uygun¹⁵, Gülsun Karasu¹⁵, Mehmet Akif Yeşilipek¹⁵, Ahmet Koç¹⁶, Erol Erduran¹⁷, Berna Atabay¹⁸, Haldun Öniz¹⁸, Hale Ören¹
¹Dokuz Eylül University Faculty of Medicine, Department of Pediatric Hematology, İzmir, Turkey
²Gazi University Faculty of Medicine, Department of Pediatric Hematology, Ankara, Turkey
³Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology, Samsun, Turkey
⁴Erciyes University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Kayseri, Turkey
⁵Ankara University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Ankara, Turkey
⁶İstanbul University Cerrahpaşa Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey
⁷Kocaeli University Faculty of Medicine, Department of Pediatric Hematology, Kocaeli, Turkey
⁸Ankara Children’s Hematology and Oncology Training and Research Hospital, Ankara, Turkey
⁹Akdeniz University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Antalya, Turkey
¹⁰Dr. Behçet Uz Children Training and Research Hospital, Clinic of Pediatric Hematology and Oncology, İzmir, Turkey
¹¹Necmettin Erbakan University Meram Faculty of Medicine, Department of Pediatric Hematology, Konya, Turkey
¹²Uludağ University Faculty of Medicine, Department of Pediatric Hematology, Bursa, Turkey
¹³Ege University Faculty of Medicine, Department of Pediatric Hematology, İzmir, Turkey
¹⁴Şişli Hamidiye Etfal Training and Research Hospital, Clinic of Pediatric Hematology and Oncology, İstanbul, Turkey
¹⁵Bahçeşehir University Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey
¹⁶Marmara University Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey
¹⁷Karadeniz Technical University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Trabzon, Turkey
¹⁸Tepecik Training and Research Hospital, Clinic of Pediatric Hematology and Oncology, İzmir, Turkey

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.

Keywords: Hematopoietic stem cell transplantation, Juvenile myelomonocytic leukemia, Turkey

Özlem Tüfekçi, Ülker Koçak, Zühre Kaya, İdil Yenicesu, Canan Albayrak, Davut Albayrak, Şebnem Yılmaz Bengoa, Türkan Patıroğlu, Musa Karakükçü, Ekrem Ünal, Elif Ünal İnce, Tali İleri, Mehmet Ertem, Tiraje Celkan, Gül Nihal Özdemir, Nazan Sarper, Dilek Kaçar, Neşe Yaralı, Namık Yaşar Özbek, Alphan Küpesiz, Tuba Karapınar, Canan Vergin, Ümran Çalışkan, Hüseyin Tokgöz, Melike Sezgin Evim, Birol Baytan, Adalet Meral Güneş, Deniz Yılmaz Karapınar, Serap Karaman, Vedat Uygun, Gülsun Karasu, Mehmet Akif Yeşilipek, Ahmet Koç, Erol Erduran, Berna Atabay, Haldun Öniz, Hale Ören. Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. Turk J Hematol. 2018; 35(1): 27-34

Sorumlu Yazar: Özlem Tüfekçi, Türkiye

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