POST–ESSENTIAL THROMBOCYTHEMIA AND POST–POLYCYTHEMIA VERA
POSTER 14
MYELOFIBROSIS – DIFFERENCES FROM PRIMARY MYELOFIBROSIS
Bárbara Marques1,2, Rita Gomes1, Carolina Afonso1, Sara Duarte1, Adriana Roque1,2,3, Margarida
Coucelo1,3, José Pedro Carda1,2, Emília Cortesão1,2 and Letícia Ribeiro1,3
(1)Clinical Hematology Department , Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
(2)Faculty of Medicine of University of Coimbra, Coimbra, Portugal
(3)Coimbra Institute for Clinical and Biomedical Research ( iCBR ), Faculty of Medicine of University of
Coimbra, Coimbra, Portugal
Introduction and Objectives: Primary Myelofibrosis (PMF) is the most aggressive of the classic
myeloproliferative neoplasms with higher incidence to transformation to acute myeloid
leukemia (AML). It may present as de novo or post essential thrombocythemia (PET-MF) or
polycythemia vera (PPV-MF). Recently, it has been described that PET/PPV-MF may differ from
PMF. We aim to describe clinical and prognostic characteristics in both patients PET/PPV-MF
and PMF.
Methods: Retrospective analysis of patients with myelofibrosis, diagnosed according to WHO
classification criteria, between 2010 and 2019.
Results: We identified 107 patients, 69 (64.5%) males, with median age 68 years old. Sixteen
patients (15%) had PET/PPV-MF, with no differences in demographic characteristics. Median
time to progression to myelofibrosis was 5.4 years (IC95% 3.9-6.7). Patients with PET/PPV-MF
presented higher platelet counts (177 vs 233 G/L; p=0.004), with no differences in
hemoglobin, WBC, percentage of circulating blasts or lactate dehydrogenase. PET/PPV-MF
patients presented splenomegaly (45/86 52.3% vs 14/16 87.5%; p=0.012) and fibrosis ≥2
(4852.7% vs 1487.5%; p=0.012) more frequently. PET/PPV-MF patients presented systemic
symptoms (28/70 40% vs 7/13 53.8%; p=0.376) in a higher proportion of patients and less
frequent transfusion dependency (1718.9% vs 531.3%, p=0.261), although not statistically
significant. In PET/PPV-MF group, 13 patients had the JAK2 V617F mutation, with a higher
allele burden (73% vs 26; p=0.002), compared with PMF.
High risk mutations (ASXL1, EZH2, IHD1/2 and U2AF1) were tested in 29 (27.1%) patients and
were present in 6 of them (20.8% in PMF and 20% in PET/PPV-MF). Nine patients had
progressed to AML after a median time since diagnosis of 1.9 years (range, 1.4-2.5).
Although PPV-MF presented a lower median overall survival (OS) than PMF (1.5 vs 10.2 years;
HR=0.2 IC95% 0.05-0.7 p=0.165)
Conclusions: Patients with PET/PPV-MF present more frequently splenomegaly, fibrosis and a
higher JAK2 V617F allele burden when compared with PMF. Although the frequency of high
risk mutations and unfavorable cytogenetic abnormalities were similar among the two groups,
PPV-MF patients had lower OS. In patients with secondary MF the identification of prognostic
factors is essential to better guide clinical decision.
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