All we know about polycythemia vera: literature review and own experience

Абдулкадыров, К М; Shuvaev, Vasiliy; Martynkevich, Irina

doi:10.17650/1818-8346-2015-10-3-28-42

Cited by 6 publications

(4 citation statements)

References 58 publications

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“…При длительном течении заболевания может происходить развитие миелофиброза или бластной трансформации. Частым клиническим проявлением МПН, особенно ИП и ЭТ, является развитие тромботических осложнений [1]. Частота артериальных и венозных тромбозов у больных ИП и ЭТ составляет 2,62 и 1,77 % в год соответ-ственно, что в 1,5 и 3,2 раза выше, чем у населения в целом [2].…”

Section: резюмеunclassified

Global tests in patients with Ph-negative myeloproliferative neoplasms

Silina,

Korsakova,

Golovina

et al. 2023

Gematologiâ i transfuziologiâ

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Introduction: A frequent clinical manifestation of Ph-negative myeloproliferative neoplasms (MPN) is the development of thrombosis. To identify the state of hypercoagulation it is relevant and promising to introduce global tests for evaluating the hemostasis — the thrombin generation test (TGT) and thromboelastography (TEG). Aim: to evaluate the parameters of thrombin generation and thromboelastography in patients with Ph-negative MPN. Material and methods. In total, 62 patients with MNP were included in the study: 27 with polycythemia vera (PV), 14 with essential thrombocythemia (ET) and 21 with primary myelofibrosis (PMF). The control group included 55 practically healthy individuals, comparable in gender and age (19 people in the study of TEG, 36 people in the study of TGT). The TEG was performed using a “TEG 5000” thromboelastograph. TGT was measured with Calibrated Automated Thrombinography. Results: Ly30 and Ly60 in TEG in patients were significantly lower (0.35 (0.20–0.48), 0.00 (0.00–0.40) and 0.00 (0.00 — 0.43) and 3.15 (2.45–3.60), 1.25 (0.10–3.58) and 0.60 (0.00–3.05) respectively), than in the control (1.60 (1.05–2.75) and 6.20 (4.15–8.30), respectively), which indicates the ineffectiveness of fibrinolysis. The values of MA and G in patients with ET and PV significantly exceeded the control (69.15 (67.98–70.78) mm and 65.20 (59.65–63.83) mm versus 62.00 (57.75–6.75) mm and 11.20 (10.60–12.15) din/cm2 and 9.40 (7.40–11.60) din/cm2 versus 8.20 (6.85–8.75) din/cm2, respectively. The most pronounced change in sensitivity to TM was observed in patients with ET (27.94 (17.35–43.58) % and 13.29 (-3.48–23.60) %, respectively; p < 0.05). A significant decrease in ETP was observed in patients with PV and PMF. Conclusion. The study of hemostasis in patients with MPN using TEG and TGT revealed the presence of multidirectional changes associated with the disease. The TEG showed that an increase in the time required for the onset of fibrin formation is combined with increased clot strength and inhibited fibrinolysis, which are risk factors for the development of thromboembolic complications. The study of TGT determined a decrease in the quantitative characteristics of thrombin generation and at the same time the failure of the anticoagulant system of protein C, leading to the development of hypercoagulation.

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Section: резюмеunclassified

Global tests in patients with Ph-negative myeloproliferative neoplasms

Silina,

Korsakova,

Golovina

et al. 2023

Gematologiâ i transfuziologiâ

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“…We have applied different treatment algorithms to every Ph-MPN form (ET, PV, PMF), based on risks for survival and/ or thrombosis [7][8][9]. .…”

Section: Ocimum Scientific Publishersmentioning

confidence: 99%

“…In case of intermediate age patients with significantly cardiovascular diseases the treatment was similar with exception that flebotomy/red cell apheresis were tried to be avoided. Older (>70 years) patients were treated with HU as choice for first line, in case of resistance and/or intolerance the RUX or other chemotherapeutic agents or IFN were proposed[7].…”

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confidence: 99%

Implementation of Algorithm-Based Approach for Ph-Negative Myeloproliferative Neoplasms in Routine Clinical Practice in Russia

Ltd¹

2019

DTHJ

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Background: In recent years there has been significant progress in the diagnostic and treatment of Ph-negative myeloproliferative neoplasms. By the way, they remain heterogenous with great variability in clinical course for every patient. The individual choice from several available treatment methods should be based on balance between potential benefit and risk of adverse events and disease progression. There are some prognostic models for every classic Ph-negative myeloproliferative disease (Essential Thrombocythemia, Polycythemia Vera and Primary Myelofibrosis) that based on retrospective analysis. The implications of treatment algorithms based on such prognostic scales could apply for Phnegative myeloproliferative neoplasms in routine clinical practice.Aim: To compare results of diagnostic and treatment algorithms for Ph-MPN patients management with previous experience. Materials and methods:A database of Ph-MPN patients study group (97 ET patients, 174 PV patients, 94 PMF patients) and control group (116 ET patients, 265 PV patients, 182 PMF patients). Characteristics of the groups of patients were similar. The diagnosis and treatment of study group patients was conducted according to own risk-based algorithms since year 2012. We have applied different treatment algorithms to every Ph-MPN form (ET, PV, PMF), based on risks for survival and/or thrombosis. Results:The implementation of diagnostic algorithm diminished the period between first hematologist referral to diagnosis established from 57 days in control group up to 16 days in study group. The use of risk-adapted approach led to two-times more frequent ET patient's management without cytoreductive therapy (32% in study group vs 14% in control group). Five-year thrombosisfree survival was 91% in study group and 79% in control group. Relative risk for thrombosis was 0.34; 95% C.I. 0.15-0.81; p=0.01). Our ET management algorithm allowed to decrease thrombosis probability from 0.039 event per patient-year in control group to 0.032 event per patient-year in study group. Overall 5-year survival was 93% in study group and 80% in control group. The death probability was decreased from 0.026 event per patient-year in control group to 0.018 event per patient-year. The PV management was also improved with implementation of risk-based algorithm. The cytoreduction in total (drug + excessive red cell removal) were significantly (p=0.0001) rarer in study group (87%) than in control group (98%). HU was used significantly (p=0.0001) more frequent in control group (83%) than in study group (67%). HU as only monotherapy was significantly (p=0.0001) more frequently used in study group (24%) than in control group (11%). Despite of the rarer use of cytoreduction the complete responses almost two times were significantly (p=0.004) frequently

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“…Hypermethylation of CpG islands associated with promoters of tumor suppressor genes and, more recently, microRNA [15][16][17], leading to gene silencing and thus inactivation of tumor suppressor genes, has been linked to the pathogenesis of hematologic malignancies [18]. Furthermore, aberrant DNA methylation of SOCS1, SOCS2, SOCS3, and SHP1 was investigated in Phve MPN with conflicting results [19][20][21][22][23]. A previous study has shown that while normal cells have unmethylated CpG islands around the transcription start site (TSS) at the 5'UTR, CpG sites within the exon coding sequence for SOCS1 are methylated, resulting in indicates the importance of the specific polymerase chain of methylation.…”

Section: Introductionmentioning

confidence: 99%

Methylation Profiling SOCS2 in Philadelphia-Negative Myeloproliferative Neoplasm

Yasin

2021

JPRI

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The Janus kinase signal transducer and activator of transcription (JAK / STAT) signalling, which is crucial in Philadelphia-negative myeloproliferative neoplasms’ (MPNs), is negatively regulated by molecules such as SOCS, CISH, and SHP1. SOCS2 methylation has been studied in MPN with conflicting results. Here we examine the methylation status of SOCS2 by specific methylation-specific polymerase chain reaction (MSP) in cell lines and 130 diagnostic prepheral blood samples from Ph-ve MPN. Furthermore, we tried to explain the mismatch of the methylation frequency by assigning the investigated MSP primers to the respective genes. Methylation was detected in normal controls using SOCS2-MSP primers in the 3 'translated exon sequence, but not with primers around the transcription start site in the 5' untranslated regions (5'UTR). SOCS2 was completely unmethylated in primary MPN samples and cell lines. In contrast, SOCS2 methylation when using MSP primers located at the 5'UTR is rare in all studies. In conclusion, SOCS2 methylation is rare in Ph-ve MPN. The appropriate MSP primers are important for an accurate estimate of the methylation frequency. The role of SOCS2 methylation in MPN pathogenesis requires further investigation.

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All we know about polycythemia vera: literature review and own experience

Cited by 6 publications

References 58 publications

Global tests in patients with Ph-negative myeloproliferative neoplasms

Global tests in patients with Ph-negative myeloproliferative neoplasms

Implementation of Algorithm-Based Approach for Ph-Negative Myeloproliferative Neoplasms in Routine Clinical Practice in Russia

Methylation Profiling SOCS2 in Philadelphia-Negative Myeloproliferative Neoplasm

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