title: Interferon α-2b gains high sustained response therapy for advanced essential thrombocythemia and polycythemia vera with JAK2V617F positive mutation.
abstract: OBJECTIVE
  This open-label, prospective, observational study aimed to evaluate treatment response, efficacy therapy and safety to IFN α-2b for the essential thrombocythemia (ET) and polycythemia vera (PV) with JAK2V617F positive mutation.
  
  
  METHODS
  A total of 123 ET patients received IFNα-2b therapy with JAK2V617F positive or negative mutation; and 136 PV patients with JAK2V617F(+) received IFNα-2b or hydroxyurea (HU) therapy according to random number assignment (ages 18-65 years old).
  
  
  RESULTS
  ET patients receiving IFN α-2b with JAK2V617F(+) had a greater advantage in overall hematologic response (OHR) than JAK2V617F(-) (83.3% versus 61.4%, P<0.01). For PV patients with JAK2V617F(+), IFN had no OHR superiority to HU (70.3% versus 70.8%, P>0.05), but which gained a greater satisfactory molecular response than HU (54.7% versus 19.4%, P<0.01). IFN significantly decreased the phlebotomy rate, which was better than HU for MPDs patients with OHR than HU (3.6% versus 65.7%, P<0.01). Furthermore, ET patients with JAK2V617F(+) demonstrated a definite advantage over JAK2V617F(-) in five-year PFS (75.9% versus 47.6%, P<0.05). For PV patients with JAK2V617F(+), IFN α-2b was superior to HU in five-year PFS (66.3% versus 46.7%, P<0.01). Moreover, IFN α-2b also contributed to improved vasomotor symptoms in MPDs, and especially significantly decreased the incidence of distal paresthesias (14.1% versus 37.5%) and erythromelalgia (9.4% versus 29.2%) better than HU (P<0.01). Meanwhile, IFN did not observe the severe hematological adverse events in patients with PV or ET.
  
  
  CONCLUSIONS
  The data confirmed that IFN α-2b benefited the patients with ET or PV, particularly for JAK2V617F(+) mutation.
authors: Department of Hematology, The Affiliated Hospital of Inner Mongolia Medical University, 1 TongDao Avenue North, Hohhot 010059, PR China. Electronic address: huangbintao1979@sina.com.
authors: Bin-Tao
authors: BT
authors: Huang
authors: Department of Medicine, Nanfang Hospital, Southern Medical University, 1838 North Guangzhou Ave, Guangzhou, Guangdong 510515, PR China.
authors: Qing-Chun
authors: QC
authors: Zeng
authors: Department of Gastroenterology, The Affiliated Hospital of Inner Mongolia Medical University, 010059 Hohhot, PR China.
authors: Wei-Hong
authors: WH
authors: Zhao
authors: Huizhou Medicine Institute, 516003 Huizhou, PR China.
authors: Bing-Sheng
authors: BS
authors: Li
authors: Department of Medicine, The Second Affiliated Hospital of Guangzhou Medical University, 510260 Guangzhou, China.
authors: Rui-Lin
authors: RL
authors: Chen
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D006918:Hydroxyurea; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use; D053614:Janus Kinase 2 / Q000235:genetics; D053208:Kaplan-Meier Estimate; D009154:Mutation; D011087:Polycythemia Vera / Q000188:drug therapy / Q000235:genetics / Q000401:mortality; D011994:Recombinant Proteins / Q000627:therapeutic use; D013920:Thrombocythemia, Essential / Q000188:drug therapy / Q000235:genetics / Q000401:mortality
chemical_list: D006918:Hydroxyurea; D000077190:Interferon alpha-2; D016898:Interferon-alpha; C507924:JAK2 protein, human; D053614:Janus Kinase 2; D011994:Recombinant Proteins
keywords: Essential thrombocythemia; Interferon; JAK2V617F mutation; Polycythemia vera