Disease and Clinical Characteristics of Patients with Chronic Myeloproliferative Neoplasms: 11-year single center experience

INTRODUCTION: BCR/ABL-negative myeloproliferative neoplasms are characterized by over-production myeloid lineages in the bone marrow. Polycythemia vera, essential thrombocythemia and primary myelofibrosis are the most common myeloproliferative neoplasms. The aim of this study was to analyse patient demographic characteristics, laboratory findings, mutational status together with complications, clinical course and survival.
METHODS: This study was conducted on patients diagnosed with myeloproliferative neoplasms between 2008 and 2019. Blood parameters, demographic information, mutation analysis, management, complications and follow-up periods were recorded. Survival rates were calculated and the effect of the parameters on overall survival was analyzed.
RESULTS: Evaluation was made of 247 patients, comprising 105 polycythemia vera, 126 essential thrombocythemia and 16 primary myelofibrosis patients. During follow-up, 11 polycythemia vera, 14 essential thrombocythemia and 2 primary myelofibrosis patients developed thromboembolic complications. Median overall survival could not be reached in polycythemia vera and essential thrombocythemia patient and determined as 70.3 months in primary myelofibrosis patients. Age, LDH, ferritin and platelet/lymphocyte ratio at the time of diagnosis and thromboembolic complications were determined to have a statistically significant effect on survival in all patients. Lower survival rates were seen in the primary myelofibrosis patients although thromboembolic complications were observed at similar rates in all 3 disease subgroups.

DISCUSSION AND CONCLUSION: In addition to known risk factors such as age and thromboembolic complications, parameters such as LDH, ferritin and PLR, which may be considered to indicate disease activity and inflammation, can also be used as prognostic markers.

Kronik Myeloproliferatif Neoplazi Tanýlý Hastalarda Klinik Özellikler ve Hastalýk Karakteristikleri: 11 yýllýk tek merkez deneyimi

GÝRÝÞ ve AMAÇ: BCR / ABL-negatif miyeloproliferatif neoplaziler, kemik iliðindeki miyeloid öncül hücrelerin aþýrý üretimi ile karakterizedir. Polisitemi vera, esansiyel trombositemi ve primer miyelofibrozis en sýk görülen miyeloproliferatif neoplazilerdir. Bu çalýþmanýn amacý hastalarýn demografik özelliklerini, klinik seyir ve laboratuvar bulgularýný komplikasyonlar ve saðkalým ile birlikte analiz etmektir.
YÖNTEM ve GEREÇLER: Bu çalýþmaya 2008-2019 yýllarý arasýnda miyeloproliferatif neoplazi tanýsý alan hastalar dahil edildi. Kan parametreleri, demografik bilgiler, mutasyon analizi, tedavi, komplikasyonlar ve takip süreleri kaydedildi. Saðkalým oranlarý hesaplandý ve parametrelerin genel saðkalým üzerindeki etkisi analiz edildi.
BULGULAR: 105 Polisitemi vera, 126 esansiyel trombositemi ve 16 primer miyelofibrozis olmak üzere oluþan toplam 247 hasta deðerlendirildi. Takip sýrasýnda 11 polisitemi vera, 14 esansiyel trombositemi ve 2 primer miyelofibrozis hastasýnda tromboembolik komplikasyon geliþti. Polisitemi vera ve esansiyel trombositemi hastalarýnda ortalama genel saðkalýma ulaþýlamadý, ancak primer miyelofibrozis hastalarýnda 70.3 ay olarak belirlendi. Taný anýnda yaþ, LDH, ferritin ve trombosit / lenfosit oraný ve tromboembolik komplikasyonlarýnýn tüm miyeloproliferatif neoplazi hastalarýnda saðkalým üzerinde istatistiksel olarak anlamlý bir etkisi olduðu belirlenmiþtir. Primer miyelofibrozis hastalarýnda daha düþük saðkalým oranlarý görülmesine raðmen, tromboembolik komplikasyon oraný 3 hasta alt grubunun hepsinde benzer oranlarda izlenmiþtir.
TARTIÞMA ve SONUÇ: Yaþ ve tromboembolik komplikasyonlar gibi bilinen risk faktörlerine ek olarak, LDH, ferritin ve PLR gibi hastalýk aktivitesini ve inflamasyonu gösteren parametreler de bu hastalýklarýn takibinde prognostik belirteç olarak kullanýlabilir.