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

Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients [Turk J Hematol]

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

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

�zlem T�fek�i¹, �lker Ko�ak², Z�hre Kaya², �dil Yenicesu², Canan Albayrak³, Davut Albayrak³, �ebnem Y�lmaz Bengoa¹, T�rkan Pat�ro�lu⁴, Musa Karak�k��⁴, Ekrem �nal⁴, Elif �nal �nce⁵, Tali �leri⁵, Mehmet Ertem⁵, Tiraje Celkan⁶, G�l Nihal �zdemir⁶, Nazan Sarper⁷, Dilek Ka�ar⁸, Ne�e Yaral�⁸, Nam�k Ya�ar �zbek⁸, Alphan K�pesiz⁹, Tuba Karap�nar¹⁰, Canan Vergin¹⁰, �mran �al��kan¹¹, H�seyin Tokg�z¹¹, Melike Sezgin Evim¹², Birol Baytan¹², Adalet Meral G�ne�¹², Deniz Y�lmaz Karap�nar¹³, Serap Karaman¹⁴, Vedat Uygun¹⁵, G�lsun Karasu¹⁵, Mehmet Akif Ye�ilipek¹⁵, Ahmet Ko�¹⁶, Erol Erduran¹⁷, Berna Atabay¹⁸, Haldun �niz¹⁸, Hale �ren¹
¹Dokuz Eyl�l University Faculty of Medicine, Department of Pediatric Hematology, �zmir, Turkey
²Gazi University Faculty of Medicine, Department of Pediatric Hematology, Ankara, Turkey
³Ondokuz May�s 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
⁶�stanbul University Cerrahpa�a Faculty of Medicine, Department of Pediatric Hematology and Oncology, �stanbul, 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. Beh�et Uz Children Training and Research Hospital, Clinic of Pediatric Hematology and Oncology, �zmir, Turkey
¹¹Necmettin Erbakan University Meram Faculty of Medicine, Department of Pediatric Hematology, Konya, Turkey
¹²Uluda� University Faculty of Medicine, Department of Pediatric Hematology, Bursa, Turkey
¹³Ege University Faculty of Medicine, Department of Pediatric Hematology, �zmir, Turkey
¹⁴�i�li Hamidiye Etfal Training and Research Hospital, Clinic of Pediatric Hematology and Oncology, �stanbul, Turkey
¹⁵Bah�e�ehir University Faculty of Medicine, Department of Pediatric Hematology and Oncology, �stanbul, Turkey
¹⁶Marmara University Faculty of Medicine, Department of Pediatric Hematology and Oncology, �stanbul, 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, �zmir, 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

T�rkiye�de Juvenil Miyelomonositik L�semi: Altm�� Be� Hastan�n Retrospektif Analizi

Ama�: T�rkiye�deki juvenil miyelomonositik l�semi (JMML) hastalar�n�n durumunu, tan� zaman�, klinik �zellikler, mutasyon �al��malar�, klinik gidi� ve tedavi stratejileri a��s�ndan ortaya koymakt�r.
Gere� ve Y�ntem: �lkemizdeki pediatrik hematoloji ve onkoloji kliniklerinden veri istenerek, JMML tan�s� ile takip ve tedavisi yap�lan hastalar�n klinik ve laboratuvar bulgular� geriye d�n�k olarak de�erlendirildi.
Bulgular: On sekiz merkezden, 2002-2016 tarihleri aras�nda JMML tan�s� alan toplam 65 hasta �al��maya dahil edildi. Ortanca tan� ya�� 17 ay idi (2-117 ay). Splenomegali tan�da %92 hastada vard�. Ortanca l�kosit, monosit ve trombosit say�lar� s�ras�yla 32,9x109/L, 5,4x109/L ve 58,3x109/L idi. Monozomi 7, %18 hastada saptanm��t�. JMML mutasyonlar� 32 hastada (%49) �al��lm�� olup, en s�k rastlanan mutasyon PTPN11 idi. Hematopoetik k�k h�cre nakli (HKHN) hastalar�n ancak %44��ne uygulanabilmi� olup, nakillerin b�y�k bir oran� 2012 y�l�ndan sonra yap�lm��t�. Nakil yap�lamamas�n�n en s�k nedeni uygun don�r bulunamamas�yd�. Tan� ald�ktan nakile kadar ge�en ortalama s�re 9 ay (2-63 ay) olarak saptand�. T�m hastalarda 5 y�ll�k k�m�latif sa�kal�m oran� %33, ortanca tahmini ya�am s�resi ise 30�17,4 ay (%95 CI: 0-64,1) olarak bulundu. Sa�kal�m s�resi HKHN yap�lan hastalarda anlaml� olarak daha uzundu (log-rank p=0,019). Tan�da 2 ya��n �st�nde olmak, trombosit say�s�n�n 40x109/L�nin alt�nda saptanmas� ve PTPN11 mutasyon varl�� ya�am s�resini anlaml� olarak k�saltan fakt�rler olarak bulundu.
Sonu�: �lkemizde her ne kadar son d�nemlerde JMML hastalar�nda kesin tan� ve HKHN a��s�ndan iyile�me kaydedilmi� olsa da genel sonu� tatminkar de�ildir ve bu konu ile ilgili daha fazla �aba g�stermeye gerek vard�r.

Anahtar Kelimeler: Hematopoetik k�k h�cre nakli, Juvenil miyelomonositik l�semi, T�rkiye

�zlem T�fek�i, �lker Ko�ak, Z�hre Kaya, �dil Yenicesu, Canan Albayrak, Davut Albayrak, �ebnem Y�lmaz Bengoa, T�rkan Pat�ro�lu, Musa Karak�k��, Ekrem �nal, Elif �nal �nce, Tali �leri, Mehmet Ertem, Tiraje Celkan, G�l Nihal �zdemir, Nazan Sarper, Dilek Ka�ar, Ne�e Yaral�, Nam�k Ya�ar �zbek, Alphan K�pesiz, Tuba Karap�nar, Canan Vergin, �mran �al��kan, H�seyin Tokg�z, Melike Sezgin Evim, Birol Baytan, Adalet Meral G�ne�, Deniz Y�lmaz Karap�nar, Serap Karaman, Vedat Uygun, G�lsun Karasu, Mehmet Akif Ye�ilipek, Ahmet Ko�, Erol Erduran, Berna Atabay, Haldun �niz, Hale �ren. Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients. Turk J Hematol. 2018; 35(1): 27-34

Corresponding Author: �zlem T�fek�i, T�rkiye

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