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Guidelines for the management of myeloproliferative neoplasms
#MMPMID26552452
Choi CW
; Bang SM
; Jang S
; Jung CW
; Kim HJ
; Kim HY
; Kim SJ
; Kim YK
; Park J
; Won JH
Korean J Intern Med
2015[Nov]; 30
(6
): 771-88
PMID26552452
show ga
Polycythemia vera, essential thrombocythemia, and primary myelofibrosis are
collectively known as 'Philadelphia-negative classical myeloproliferative
neoplasms (MPNs).' The discovery of new genetic aberrations such as Janus kinase
2 (JAK2) have enhanced our understanding of the pathophysiology of MPNs.
Currently, the JAK2 mutation is not only a standard criterion for diagnosis but
is also a new target for drug development. The JAK1/2 inhibitor, ruxolitinib, was
the first JAK inhibitor approved for patients with intermediate- to high-risk
myelofibrosis and its effects in improving symptoms and survival benefits were
demonstrated by randomized controlled trials. In 2011, the Korean Society of
Hematology MPN Working Party devised diagnostic and therapeutic guidelines for
Korean MPN patients. Subsequently, other genetic mutations have been discovered
and many kinds of new drugs are now under clinical investigation. In view of
recent developments, we have revised the guidelines for the diagnosis and
management of MPN based on published evidence and the experiences of the expert
panel. Here we describe the epidemiology, new genetic mutations, and novel
therapeutic options as well as diagnostic criteria and standard treatment
strategies for MPN patients in Korea.
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