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Published on: March 10, 2023
CLINICAL AND MOLECULAR GENETIC CHARACTERISTICS OF POLYCYTHEMIA VERA AND CURRENT TREATMENT APPROACHES
F Khalilova1, A Kerimov1, G Kerimova1
1National Hematology and Blood Transfusion Center, Baku, Azerbaijan.
Masked polycythemia vera (mPV) patients experienced more thromboembolic events (TE) despite better treatment response. Timely diagnosis and treatment are crucial for mPV outcomes.
Area of Science:
- Hematology
- Oncology
- Clinical Medicine
Background:
- Polycythemia vera (PV) diagnosis relies on WHO criteria, potentially excluding patients with low hemoglobin (HGB).
- This necessitates differentiating overt PV (oPV) and masked PV (mPV) for appropriate management.
- Analyzing these forms is key to understanding disease heterogeneity.
Purpose of the Study:
- To distinguish between overt PV (oPV) and masked PV (mPV).
- To investigate thrombotic risk, JAK2V617F allele burden (AB), and treatment efficacy in oPV vs. mPV across risk groups.
- To compare thromboembolic events (TE) and hematologic response (HR) between oPV and mPV.
Main Methods:
- A cohort study with retrospective and prospective components.
- 209 PV patients were classified into oPV (136) and mPV (73) groups.
- Hemogram parameters, AB, risk stratification, TE, and HR were analyzed and compared between groups and treatment regimens.
Main Results:
- Thromboembolic events (TE) were more frequent in mPV (p<0.01).
- JAK2V617F allele burden (AB) differed significantly between oPV and mPV in the low-risk group (p=0.001).
- Masked PV (mPV) demonstrated significantly higher overall hematologic response (HR) rates compared to oPV (p = 0.003), particularly with phlebotomy/aspirin and hydroxyurea treatments.
Conclusions:
- Despite higher TE rates in mPV, potentially due to delayed diagnosis, mPV shows superior treatment response compared to oPV.
- The higher platelet (PLT) count in mPV warrants further investigation regarding thrombosis risk.
- Emphasizes the critical importance of timely and aggressive treatment for masked polycythemia vera.
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