ISSN: 1300-7777 E-ISSN: 1308-5263
Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of 65 Patients [Turk J Hematol]
Turk J Hematol. Ahead of Print: TJH-62372 | DOI: 10.4274/tjh.2017.0021  

Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of 65 Patients

Özlem Tüfekçi1, Şebnem Yılmaz Bengoa, Hale Ören1, Ülker Koçak, Zühre Kaya, İdil Yenicesu2, Canan Albayrak, Davut Albayrak3, Türkan Patıroğlu, Musa Karakükçü, Ekrem Ünal4, Elif Ünal İnce, Talia İleri, Mehmet Ertem5, Tiraje Celkan, Gül Nihal Özdemir,6, Nazan Sarper7, Dilek Kaçar, Neşe Yaralı, Namık Yaşar Özbek8, Alphan Küpesiz9, Tuba Karapınar, Canan Vergin10, Ümran Çalışkan, Hüseyin Tokgöz11, Melike Sezgin Evim, Birol Baytan, Adalet Meral Güneş12, Deniz Yılmaz Karapınar13, Serap Karaman14, Vedat Uygun, Gülsun Karasu, Mehmet Akif Yeşilipek15, Ahmet Koç16, Erol Erduran17, Berna Atabay, Haldun Öniz18
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-Oncology, Kayseri, Turkey
5Ankara University Faculty of Medicine, Department of Pediatric Hematology-Oncology, Ankara, Turkey
6İstanbul University Cerrahpaşa Faculty of Medicine, Department of Pediatric Hematology-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-Oncology, Antalya, Turkey
10Dr. Behçet Uz Children Training and Research Hospital, Clinic of Pediatric Hematology-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-Oncology, İstanbul, Turkey
15Bahçeşehir University Faculty of Medicine, Department of Pediatric Hematology-Oncology, İstanbul, Turkey
16Marmara University Faculty of Medicine, Department of Pediatric Hematology-Oncology, İstanbul, Turkey
17Karadeniz Technical University Faculty of Medicine, Department of Pediatric Hematology-Oncology, Trabzon, Turkey
18Tepecik Training and Research Hospital, Clinic of Pediatric Hematology-Oncology, İzmir, Turkey

Objective: 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 labarotory 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 into the study. The median age at diagnosis was 17 months (range, 2-117 months). Splenomegaly was present in 92 % of patients at the time of diagnosis. The median WBC, 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 out of 65 patients (49%), PTPN11 was the most common mutation. Hematopoetic stem cell transplantation (HSCT) could only be performed in 28 (44%) patients, majority of 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 (range,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), platelets 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 our country.

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


Türkiye’de Juvenil Miyelomonositik Lösemi: 65 Hastanın Retrospektif Analizi

Özlem Tüfekçi1, Şebnem Yılmaz Bengoa, Hale Ören1, Ülker Koçak, Zühre Kaya, İdil Yenicesu2, Canan Albayrak, Davut Albayrak3, Türkan Patıroğlu, Musa Karakükçü, Ekrem Ünal4, Elif Ünal İnce, Talia İleri, Mehmet Ertem5, Tiraje Celkan, Gül Nihal Özdemir,6, Nazan Sarper7, Dilek Kaçar, Neşe Yaralı, Namık Yaşar Özbek8, Alphan Küpesiz9, Tuba Karapınar, Canan Vergin10, Ümran Çalışkan, Hüseyin Tokgöz11, Melike Sezgin Evim, Birol Baytan, Adalet Meral Güneş12, Deniz Yılmaz Karapınar13, Serap Karaman14, Vedat Uygun, Gülsun Karasu, Mehmet Akif Yeşilipek15, Ahmet Koç16, Erol Erduran17, Berna Atabay, Haldun Öniz18
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-Oncology, Kayseri, Turkey
5Ankara University Faculty of Medicine, Department of Pediatric Hematology-Oncology, Ankara, Turkey
6İstanbul University Cerrahpaşa Faculty of Medicine, Department of Pediatric Hematology-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-Oncology, Antalya, Turkey
10Dr. Behçet Uz Children Training and Research Hospital, Clinic of Pediatric Hematology-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-Oncology, İstanbul, Turkey
15Bahçeşehir University Faculty of Medicine, Department of Pediatric Hematology-Oncology, İstanbul, Turkey
16Marmara University Faculty of Medicine, Department of Pediatric Hematology-Oncology, İstanbul, Turkey
17Karadeniz Technical University Faculty of Medicine, Department of Pediatric Hematology-Oncology, Trabzon, Turkey
18Tepecik Training and Research Hospital, Clinic of Pediatric Hematology-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-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 (2-79 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 PTPN 11 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 sağ-kalım 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 40x 109/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 miyelomonositk lösemi, Türkiye




Corresponding Author: Özlem Tüfekçi, Türkiye


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