The clinical course of essential thrombocythemia (ET) is complicated with thrombosis which significantly impacts patients' mortality. Studies have identified JAK2V617F mutation as an independent risk factor for thrombosis. Circulating extracellular vesicles (EVs) were evaluated in several studies regarding myeloproliferative neoplasms and thrombosis as potential biomarkers. The present study investigates the relationship between JAK2V617F mutation and EVs levels in 119 ET patients. Our analyses revealed that JAK2V617F-positive patients are at a significantly increased risk of thrombosis within five years before the ET diagnosis (hazard ratio [95% CI]: 11.9 [1.7-83.7], P = 0.013), and that JAK2V617F mutation is an independent risk factor for thrombosis at ET diagnosis or during the follow-up (hazard ratio [95% CI]: 3.56 [1.47-8.62], P = 0.005). ET patients have higher levels of platelet-EVs, erythrocyte-EVs and procoagulant activity of EVs than the healthy population. Absolute and relative counts of platelet-EVs are increased in the presence of JAK2V617F mutation (P = 0.018, P = 0.024, respectively). In conclusion, our results support the role of JAK2V617F mutation in the pathogenesis of thrombosis in essential thrombocythemia through enhancing platelet activation.
Trombocytopenie je běžným hematologickým nálezem v denní klinické praxi, je definována poklesem počtu trombocytů pod dolní mez normálního rozmezí. Široké spektrum příčin a různorodost klinického obrazu často představují diagnostickou výzvu. Článek je zaměřen na základní diferenciální diagnostiku trombocytopenie, což je podpořeno dvěma kazuistikami.
Thrombocytopenia is a hematological finding commonly encountered in daily clinical practice; it is defined by a decrease in the number of platelets below the lower limit of the normal range. A broad spectrum of etiologies and variation in clinical presentation often present a diagnostic challenge. The article is focused on the basic differential diagnosis of thrombocytopenia, which is supported by two case reports.
Anemie je běžná hematologická abnormalita, definovaná jako snížení koncentrace hemoglobinu pod dolní hranici normálního rozmezí. V klinické praxi je nejčastější a nejpřínosnější dělení anemií podle středního objemu erytrocytů na normocytární, mikrocytární a makrocytární. Článek je věnován základní diferenciální diagnostice anemií, která je demonstrována na příkladu několika kazuistik.
Anemia is a common hematological abnormality, defined as hemoglobin level under lower limit of normal range. In clinical practice, the most common and most beneficial division of anemia according to the mean cell volume is normocytic, microcytic and macrocytic anemias. The article is devoted to the basic differential diagnosis of anemia, which is demonstrated on the example of several case reports.
- Publikační typ
- abstrakt z konference MeSH
- Publikační typ
- abstrakt z konference MeSH
Thrombosis is the most common complication in BCR-ABL1 negative myeloproliferative neoplasms (MPN) that significantly impacts patients' mortality. Generally, there is an agreement on risk factors that possibly contribute to the increased risk of thrombosis, including age, history of thrombosis, JAK2V617F mutation, and cardiovascular risk factors. This study retrospectively investigates MPN-related and patient-related variables in relation to the thrombosis occurrence in MPN. Our analyses show that JAK2V617F-mutated patients are at a significantly increased risk of thrombosis within five years before the MPN diagnosis point with a hazard ratio (HR) of 15.49 (p=0.006). In multivariate analyses, independent risk factors for thrombotic complications during the follow-up are history of thrombosis (HR=2.23, p=0.019), age over 60 years at diagnosis (HR=1.56, p=0.037), the presence of JAK2V617F mutation (HR=3.01, p=0.002), and tobacco smoking (HR=1.75, p=0.01). Our results support the multifactorial mechanism of thrombosis in MPN patients, which demands individual and complex management.
- MeSH
- Janus kinasa 2 genetika MeSH
- lidé středního věku MeSH
- lidé MeSH
- mutace MeSH
- nádory * MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- trombóza * genetika MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
BACKGROUND/AIM: This work aimed to prospectively evaluate the clinical significance of circulating microparticles (MPs) in relation to thrombotic risk factors and thrombotic complications in patients with BCR/ABL1-negative myeloproliferative neoplasms (MPN). PATIENTS AND METHODS: In a cohort of 206 patients with MPN, MPs' procoagulant activity was measured by the Zymuphen functional assay in 429 samples, while platelet- and erythrocyte-MPs were enumerated by flow cytometry in 558 samples. RESULTS: MPN patients had higher MP levels than the control group. The levels of MPs were higher in male patients, smokers, and those who were older than 60 years, and in the presence of JAK2V617F mutation, history of thrombosis, platelets >400×109/l, hematocrit >45%, or leukocytes >10×109/l. Cytoreductive treatment reduced MP levels, with anagrelide being associated with lower MP levels than hydroxyurea. CONCLUSION: The relationship with thrombotic risk factors indicates a possible role of MPs in the complex thrombotic mechanism, though cytoreductive treatment seems to affect this role through reducing MP levels.
- MeSH
- lidé MeSH
- mikropartikule * MeSH
- myeloproliferativní poruchy * farmakoterapie genetika MeSH
- nádory * MeSH
- trombocyty MeSH
- trombóza * etiologie genetika MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Publikační typ
- abstrakt z konference MeSH
- MeSH
- anemie * chemicky indukované MeSH
- klinické lékařství MeSH
- lékařská onkologie MeSH
- lidé MeSH
- protokoly protinádorové léčby MeSH
- transfuze erytrocytů MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
