TY - JOUR
T1 - Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study
AU - Campbell, PJ
AU - Scott, LM
AU - Buck, G
AU - Wheatley, Keith
AU - East, CL
AU - Marsden, JT
AU - Duffy, A
AU - Boyd, EM
AU - Bench, AJ
AU - Scott, MA
AU - Vassiliou, GS
AU - Milligan, Donald
AU - Smith, SR
AU - Erber, WN
AU - Bareford, D
AU - Wilkins, BS
AU - Reilly, JT
AU - Harrison, CN
AU - Green, AR
PY - 2005/12/9
Y1 - 2005/12/9
N2 - BACKGROUND: An acquired V617F mutation in JAK2 occurs in most patients with polycythaemia vera, but is seen in only half those with essential thrombocythaemia and idiopathic myelofibrosis. We aimed to assess whether patients with the mutation are biologically distinct from those without, and why the same mutation is associated with different disease phenotypes. METHODS: Two sensitive PCR-based methods were used to assess the JAK2 mutation status of 806 patients with essential thrombocythaemia, including 776 from the Medical Research Council's Primary Thrombocythaemia trial (MRC PT-1) and two other prospective studies. Laboratory and clinical features, response to treatment, and clinical events were compared for V617F-positive and V617F-negative patients with essential thrombocythaemia. FINDINGS: Mutation-positive patients had multiple features resembling polycythaemia vera, with significantly increased haemoglobin (mean increase 9.6 g/L, 95% CI 7.6-11.6 g/L; p
AB - BACKGROUND: An acquired V617F mutation in JAK2 occurs in most patients with polycythaemia vera, but is seen in only half those with essential thrombocythaemia and idiopathic myelofibrosis. We aimed to assess whether patients with the mutation are biologically distinct from those without, and why the same mutation is associated with different disease phenotypes. METHODS: Two sensitive PCR-based methods were used to assess the JAK2 mutation status of 806 patients with essential thrombocythaemia, including 776 from the Medical Research Council's Primary Thrombocythaemia trial (MRC PT-1) and two other prospective studies. Laboratory and clinical features, response to treatment, and clinical events were compared for V617F-positive and V617F-negative patients with essential thrombocythaemia. FINDINGS: Mutation-positive patients had multiple features resembling polycythaemia vera, with significantly increased haemoglobin (mean increase 9.6 g/L, 95% CI 7.6-11.6 g/L; p
UR - http://www.scopus.com/inward/record.url?scp=28244442441&partnerID=8YFLogxK
U2 - 10.1016/S0140-6736(05)67785-9
DO - 10.1016/S0140-6736(05)67785-9
M3 - Article
C2 - 16325696
SN - 1474-547X
VL - 366
SP - 1945
EP - 1953
JO - Lancet
JF - Lancet
ER -