Multicenter Retrospective Analysis of Turkish Patients with Chronic Myeloproliferative NeoplasmsNur Soyer1, İbrahim C Haznedaroğlu2, Melda Cömert1, Demet Çekdemir3, Mehmet Yılmaz4, Ali Ünal5, Gülsüm Çağlıyan6, Oktay Bilgir6, Osman İlhan7, Füsun Özdemirkıran8, Emin Kaya9, Fahri Şahin1, Filiz Vural1, Güray Saydam11Ege University Faculty of Medicine, Department of Hematology, İzmir, Turkey
2Hacettepe University Faculty of Medicine, Department of Hematology, Ankara, Turkey
3Sakarya University Training and Research Hospital, Clinic of Hematology, Sakarya, Turkey
4Gaziantep University Faculty of Medicine, Department of Hematology, Gaziantep, Turkey
5Erciyes University Faculty of Medicine, Department of Hematology, Kayseri, Turkey
6İzmir Bozyaka Training and Research Hospital, Clinic of Hematology, İzmir, Turkey
7Ankara University Faculty of Medicine Hospital, Department of Hematology, Ankara, Turkey
8İzmir Atatürk Training and Research Hospital, Clinic of Hematology, İzmir, Turkey
9İnönü University Faculty of Medicine Hospital, Department of Hematology, Malatya, Turkey
Objective: Chronic myeloproliferative neoplasms (CMPNs) that include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) are Philadelphia-negative malignancies characterized by a clonal proliferation of one or several lineages. The aim of this report was to determine the demographic features, disease characteristics, treatment strategies, and survival rates of patients with CMPNs in Turkey.
Materials and Methods: Across all of Turkey, 9 centers were enrolled in the study. We retrospectively evaluated 708 CMPN patients’ results including 390 with ET, 213 with PV, and 105 with PMF.
Results: The JAK2V617F mutation was found positive in 86% of patients with PV, in 51.5% of patients with ET, and in 50.4% of patients with PMF. Thrombosis and bleeding at diagnosis occurred in 20.6% and 7.5% of PV patients, 15.1% and 9% of ET patients, and 9.5% and 10.4% of PMF patients, respectively. Six hundred and eight patients (85.9%) received cytoreductive therapy. The most commonly used drug was hydroxyurea (89.6%). Leukemic and fibrotic transformations occurred at rates of 0.6% and 13.2%. The estimated overall survival in PV, ET, and PMF patients was 89.7%, 85%, and 82.5% at 10 years, respectively. There were no significant differences between survival in ET, PV, and PMF patients at 10 years.
Conclusion: Our patients’ results are generally compatible with the literature findings, except for the relatively high survival rate in PMF patients. Hydroxyurea was the most commonly used cytoreductive therapy. Our study reflects the demographic features, patient characteristics, treatments, and survival rates of Turkish CMPN patients.
Keywords: Chronic myeloproliferative neoplasms, Treatment, Survival, JAK2 mutation
Kronik Miyeloproliferatif Neoplazi Tanılı Türk Hastaların Geriye Dönük ve Çok Merkezli AnaliziNur Soyer1, İbrahim C Haznedaroğlu2, Melda Cömert1, Demet Çekdemir3, Mehmet Yılmaz4, Ali Ünal5, Gülsüm Çağlıyan6, Oktay Bilgir6, Osman İlhan7, Füsun Özdemirkıran8, Emin Kaya9, Fahri Şahin1, Filiz Vural1, Güray Saydam11Ege University Faculty of Medicine, Department of Hematology, İzmir, Turkey
2Hacettepe University Faculty of Medicine, Department of Hematology, Ankara, Turkey
3Sakarya University Training and Research Hospital, Clinic of Hematology, Sakarya, Turkey
4Gaziantep University Faculty of Medicine, Department of Hematology, Gaziantep, Turkey
5Erciyes University Faculty of Medicine, Department of Hematology, Kayseri, Turkey
6İzmir Bozyaka Training and Research Hospital, Clinic of Hematology, İzmir, Turkey
7Ankara University Faculty of Medicine Hospital, Department of Hematology, Ankara, Turkey
8İzmir Atatürk Training and Research Hospital, Clinic of Hematology, İzmir, Turkey
9İnönü University Faculty of Medicine Hospital, Department of Hematology, Malatya, Turkey
Amaç: Polisitemia vera (PV), esansiyel trombositemi (ET) ve primer miyelofibrozu (PMF) içeren kronik miyeloproliferatif neoplaziler (KMPN), bir ya da birden fazla serinin klonal proliferasyonu ile karakterize Philadelphia kromozomu negatif olan malignitelerdir. Bu çalışmanın amacı, Türkiye’de KMPN’li hastaların demografik özellikleri, hastalık karakteristikleri, tedavi stratejileri ve yaşam oranlarını belirlemektir.
Gereç ve Yöntem: Türkiye’nin her yanından 9 merkez çalışmaya katıldı. Biz geriye dönük olarak ET’li 390, PV’li 213 ve PMF’li 105 hasta olmak üzere toplam 708 KMPN’li hastanın verisini değerlendirdik.
Bulgular: JAK-2 mutasyonu PV’li hastaların %86’sında, ET’li hastaların %51,5’inde ve PMF’li hastaların %50,4’ünde pozitif bulundu. Tanıda tromboz ve kanama, PV’li hastaların sırasıyla %20,6 ve %7,5’inde, ET’li hastaların %15,1 ve %9’unda ve PMF’li hastaların %9,5 ve %10,4’ünde saptandı. Altı yüz sekiz hasta (%85,9) sitoredüktif tedavi almıştı. En sık kullanılan ilaç hidroksiüre (%89,6) idi. Lösemik ve fibrotik transformasyon sıklığı %0,6 ve %13,2 idi. 10 yıllık hesaplanan toplam sağkalım PV, ET ve PMF hastalarında sırasıyla %89,7, %85 ve %82,5 idi. 10 yıllık toplam sağkalım açısından ET, PV ve PMF hastalarında anlamlı fark yoktu.
Sonuç: Sonuçlarımız, PMF hastalarının yüksek sağkalımı hariç literatürle benzerdir. Hidroksiüre ülkemizdeki en sık kullanılan sitoredüktif ajandır. Bizim çalışmamız, Türk KMPN hastalarının demografik özelliklerini, hastaların karakteristiklerini, tedavilerini ve sağkalım oranlarını yansıtmaktadır.
Anahtar Kelimeler: Kronik miyeloproliferatif neoplaziler, Tedavi, Sağkalım, JAK2 mutasyonu
Nur Soyer, İbrahim C Haznedaroğlu, Melda Cömert, Demet Çekdemir, Mehmet Yılmaz, Ali Ünal, Gülsüm Çağlıyan, Oktay Bilgir, Osman İlhan, Füsun Özdemirkıran, Emin Kaya, Fahri Şahin, Filiz Vural, Güray Saydam. Multicenter Retrospective Analysis of Turkish Patients with Chronic Myeloproliferative Neoplasms. Turk J Hematol. 2017; 34(1): 27-33
Corresponding Author: Nur Soyer, Türkiye |
|