Multicenter Retrospective Analysis of Turkish Patients with Chronic Myeloproliferative Neoplasms.
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.


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.


CONCLUSIONS
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.
Ege University Faculty of Medicine, Department of Hematology, İzmir, Turkey Phone: +90 232 390 42 87 E-mail: drakadnur@yahoo.com.
Nur
N
Soyer
İbrahim C
İC
Haznedaroğlu
Melda
M
Cömert
Demet
D
Çekdemir
Mehmet
M
Yılmaz
Ali
A
Ünal
Gülsüm
G
Çağlıyan
Oktay
O
Bilgir
Osman
O
İlhan
Füsun
F
Özdemirkıran
Emin
E
Kaya
Fahri
F
Şahin
Filiz
F
Vural
Güray
G
Saydam
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D044466:Asian People; D002908:Chronic Disease; D005260:Female; D006470:Hemorrhage; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D053614:Janus Kinase 2 / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009196:Myeloproliferative Disorders / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D011087:Polycythemia Vera / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D012189:Retrospective Studies; D015996:Survival Rate; D013920:Thrombocythemia, Essential / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D013927:Thrombosis / Q000209:etiology; D014421:Turkey; D055815:Young Adult
D000970:Antineoplastic Agents; C507924:JAK2 protein, human; D053614:Janus Kinase 2; D006918:Hydroxyurea