ANDREAS REITER (MANNHEIM)
MANAGEMENT OF RARE TRANSLOCATIONS MPN ENTITIES FGFR1 AND JAK2
PARTNER GENERS
In the presence of eosinophilia and distinct molecular aberrations, the WHO classifies a
distinct subentity as ´myeloid/lymphoid neoplasms with eosinophilia and rearrangements of
PDGFRA, PDGFRB, FGFR1 or JAK2´. Patients with PDGFRA-/B fusion genes achieve rapid and
durable remissions on the tyrosine kinase inhibitor (TKI) imatinib. Similar to BCR-ABL positive
CML, imatinib can even be stopped after achievement of durable complete molecular
remission and encouraging treatment-free survival has recently been reported. In patients
with JAK2 and FGFR1 fusion genes, phenotype and clinical course are more aggressive and
both fusions are resistant to imatinib. Particularly in patients with FGFR1 fusions, primary
presentation in medullary or extramedullary blast phase of myeloid or lymphoid origin,
primary or early secondary resistance on or after intensive chemotherapy and rapid
progression into blast phase are frequently observed. Ruxolitinib has shown significant clinical
activity in patients with JAK2 fusions. The efficacy of ponatinib was limited in patients with
FGFR1 fusions, while preliminary data from a phase-II-trial reported achievement of complete
hematologic and cytogenetic remissions on pemigatinib. Little is yet known on long-term
efficacy of TKI in patients with JAK2 and FGFR1 fusion genes, which is why eligible patients
should be considered for allogeneic stem cell transplantation.
SCIENTIFIC PROGRAMME
SESSION I
OPTIMIZING
CYTOREDUCTION
SESSION II
MANAGEMENT OF CML
WITH TKI
SESSION III
MPN RISK
STRATIFICATION
INCLUDING VASCULAR
EVENTS
DEBATE 1
INTERFERON ALPHA
SHOULD BE FRONT LINE
THERAPY IN ALL ET/PV
PATIENTS
ROUNDTABLE 1
INFECTIONS IN
MYELOPROLIFERATIVE
DISORDERS, INCLUDING
CML
ROUNDTABLE 2
PREGNANCY AND
PARENTING
DEBATE 2
ALLOGENEIC STEM CELL
TRANSPLANTATION
SHOULD BE CONSIDERED
THIRD LINE OPTION IN
CHRONIC PHASE CML
SESSION IV
EVOLVING THERAPIES
IN MYELOFIBROSIS
SESSION V
MANAGEMENT OF
ADVANCED AND UNUSUAL
DISEASE (MPN AND CML)
SESSION VI
TREATMENT FREE
REMISSION IN CML
KEYNOTE LECTURE
SELECTED ABSTRACTS
FOR AN ORAL
PRESENTATION
SELECTED ABSTRACTS
FO R A POSTER
PRESENTATION
DISCLOSURES