information culminated with the publication of the genetically inspired prognostic scoring
system for primary and secondary MF which relies on integration of clinical, histologic, and
molecular information (MIPSS70, MIPSS70plus version2.0. Additionally, pre-PMF and ET are
characterized by similarly increased rate of thrombotic events. In a multicenter cohort of 382
pre-PMF patients collected in this study, the rate of arterial and venous thrombosis after
diagnosis was 1.0 and 0.95% patients/year. Factors significantly associated with arterial
thrombosis were age, leukocytosis, generic cardiovascular risk factors, JAK2V617F and high
molecular risk mutations, while only history of previous thrombosis, particularly prior venous
thrombosis, was predictive of venous events. The risk of total thromboses was accurately
predicted by the the international prognostic score for thrombosis in essential
thrombocythemia (IPSET) score, originally developed for ET, and corresponded to 0.67, 2.05,
and 2.95% patients/year in the low-, intermediate-, and high-risk categories. The authors
concluded that IPSET could be conveniently used for thrombosis risk stratification in patients
with pre-PMF and that it was superior to both the conventional 2-tiered score and the revised
IPSET, representing the basis for individualized management aimed at reducing the increased
risk of major cardiovascular events. A comprehensive management of pre-PMF patients
should be addressed both to improving life-expectancy and to prevent vascular complications
A key controversy is how to rationalize treatment of these patients and on what to base these
decisions. For example, in view of the adverse outcome, should they be managed as overt-
MF, applying the current IPSS or DIPSS or MIPSS70 scoring systems; or should treatment be
based more upon the clinical profile However, consensus on how to manage these patients
has not yet been reached and, indeed, frequently constitutes our main problem in daily
practice.
In April 2020 Ruxolinib was started with rapid improvement in constitutional and spleen-related
symptoms and then our patient underwent a matched, unrelated donor allogeneic
transplant. At the last follow-up (Jan 2021) she felt good with a mild skin cGVHD and with a
incomplete RBC engraftment. However, spleen was no more palpable, BM biopsy revealed a
fibrosis grade G0 and a complete molecular remission was achieved.
References:
1. Arber DA et al, Blood 2016; 127:2391-405
2. Guglielmelli P. et al. Blood 2017;129:3227-3236
3. Cervantes et al, Blood 2009;113:2895-901
4. Passamonti et al, Blood 2010; 115:1703-8
5. Gangat N et al, J Clin Oncol 2011; 29:392-7
6. Rumi Eet al. Oncotarget, 2017, Vol. 8: 101735-101744
7. Rumi E, et al. Blood. 2014 Aug 14;124(7):1062-9
8. Guglielmelli P et al., Leuk Res. 2017 Jul;58:63-72;
9. Vannucchi AM, et al. Leukemia. 2013;27:1861-9;
10. Guglielmelli P, et al. Leukemia. 2014 Sep;28(9):1804-10
11. Guglielmelli P, et al. JCO 2018; ;36(4):310-318
12. Tefferi A et al, JCO 2018; 17:1769
13. Barbui Tet al., JCO. 2011;29(23):3179;
14. Guglielmelli P et al., Blood Cancer J. 2020 Feb 25;10(2):21.
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