PERSONALIZED DIAGNOSIS AND MANAGEMENT OF BCR-ABL POSITIVE AND
POSTER 17
NEGATIVE CHRONIC MYELOPROLYPHERATIVE NEOPLASMS
Vasile Musteata, Ivan Negara and Larisa Musteata
State University of Medicine and Pharmacy "N. Testemitanu", Institute of Oncology, Chisinau, Moldova,
The Republic of
Background: Chronic myeloproliferative neoplasms (CMPN) are the clonal malignancies of
hematopoietic system with primary bone marrow involvement, which comprise roughly 40%
of leukemia cases in adults, being characterized in the advanced stages by a severe, recurrent
evolution and negative socio-economic impact.
Objectives: The study aim was to assess the patterns of clinical, hematological, molecular-genetic
diagnosis and treatment options in CMPN under the personalized management
orderliness.
Methods: The study enrolled 247 patients with different phases of primary myelofibrosis
(PMF), chronic myeloid leukemia (CML) and polycythemia vera (PV), aged 14 – 81 years, who
were treated and followed up at the Institute of Oncology between 1995 – 2020. The
commonly used research methods were: clinico-analytical, epidemiological, descriptive and
cohort statistics. The diagnosis of CMPN was proved by histopathological, cytological,
molecular examinations and FISH. TKIs were administered in the newly diagnosed CML
patients, in cases of resistance to non-TKIs chemotherapy and interferon-α. PV and PMF
patients were treated with busulfan, hydroxycarbamide and interferon-α, combined with
phlebotomies in PV.
Results: Of the CMPN totality, there were 30 (12.1%) patients with PMF, 125 (50.6%) patients
with CML and 92 (37.3%) - with PV. The diagnosis of CMPN was proved mostly in the advanced
phases due to the latent, insidious onset, delayed addressability of patients and deficient
screening at the level of primary medical care. The personalized approach to the actual issues
of diagnosis and management of CMPN allowed identification of certain clinical,
hematological, cytogenetic and molecular correlations. The association of splenomegaly and
hepatomegaly was registered more frequently in PMF patients. Plethoric syndrome occurred
in 92 (100%) patients with PV and in 9 (30%) patients with Vaughan type of PMF, being more
significant in PV. In CML the rate of BCR-ABL p210 and p190 transcripts varied between 21.84-
100%, and exceeded 65% in the majority of patients (69.8%). In PV patients the 5- and 10-year
overall survival (OS) was 100% and 85.2% under the chemotherapy with hydroxycarbamide,
without significant difference to the same parameters under the chemotherapy with busulfan
(97.4% and 86.9%). Two (2.17%) relapsed and refractory PV patients underwent interferon-α
immunotherapy and one (1.09%) - anagrelide medication, with long-lasting complete
remissions for 61-79 months. The complete molecular response was achieved in 26.3% of CML
cases under the treatment with TKIs. In the elderly CML patients the 1- and 5-year OS was
97.6% and 79.8%, didn't much differ from the same parameters in the CML totality (98.5% and
87%), and in both genders turned to be superior to those obtained under the conventional
chemotherapy and α-interferon. Regardless the age and gender, in PMF patients the remission
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