Cyclic thrombocytopenia (CTP) is a rare disease with less than 70 reported cases. The hallmark of CTP is a periodic fluctuation of the platelet count. The severity of thrombocytopenia and the duration of cycles may vary.
We evaluated coagulation abnormalities via traditional tests and rotational thromboelastometry (ROTEM) in a group of 94 patients with confirmed SARS-CoV-2 infection and different severity of pneumonia (34 moderate, 25 severe, 35 critical) with the hypothesis that ROTEM parameters differed by coronavirus disease 2019 (COVID-19) severity. Shorter than normal clotting time (CT) and higher than normal maximum clot firmness (MCF) in extrinsic rotational thromboelastometry (EXTEM) and fibrinogen rotational thromboelastometry (FIBTEM), shorter than normal EXTEM clot formation time (CFT), and higher than normal α-angle were classified as markers of hypercoagulable state. Increment in the number of patients with ≥2 hypercoagulable parameters, higher EXTEM (P = .0001), FIBTEM MCF (P = .0001) and maximum lysis decrement (P = .002) with increment in disease severity was observed (P = .0001). Significant positive correlations between IL6 and CT EXTEM (P = .003), MCF EXTEM (P = .033), MCF FIBTEM (P = .01), and negative with ML EXTEM (P = .006) were seen. Our findings based on analysis of different disease severity groups confirmed that a hypercoagulable ROTEM pattern characterized by clot formation acceleration, high clot strength, and reduced fibrinolysis was more frequent in advanced disease groups and patients with high IL6. These results supported the need for different thromboprophylaxis approaches for different severity groups.
High-hemorrhagic early death (ED) rate is a major impediment in the managing of acute promyelocytic leukemia (APL). In our group of 56 newly diagnosed APL patients, ED occurred in 12 subjects, due to endocranial bleeding (8/12), differentiation syndrome (2/12), or infection (2/12). Predictors of hemorrhagic ED were as follows: white blood cells count ≥ 20 × 10(9)/L (P = 0.002337), Eastern cooperative oncology group performance status ≥ 3 (P = 0.00173), fibrinogen level <2 g/L (P = 0.004907), prothrombin time <50% (P = 0.0124), and International Society of Thrombosis and Hemostasis Scoring System for disseminated intravascular coagulation (ISTH DIC score) ≥ 6 (P = 0.00741). Multivariate analysis indicated ISTH DIC score ≥ 6 to be the most significant predictor for hemorrhagic ED (P = 0.008). The main finding of this study is that simple coagulation-related tests, performed on hospital admission and combined in the ISTH DIC score, might help to identify patients at high risk for fatal bleeding needing more aggressive supportive measures.
The current widely accepted stratification defined by age and previous thrombosis in patients with essential thrombocythemia (ET) probably deserves deeper analysis. The aim of our study was to identify additional factors at the time of diagnosis, which have an impact on the thrombosis prediction. We conducted a study of 244 consecutive ET patients with median follow-up of 83 months. We analyzed the influence of age, gender, laboratory parameters, history of previous thrombosis, spleen size, JAK2 mutation as well as cardiovascular (CV) risk factors including arterial hypertension, diabetes, active tobacco use and hyperlipidemia in the terms of thrombosis. The most important predictors of thrombosis in multivariate Cox regression model were the presence of CV risk factors (p=0.004) and previous thrombosis (p=0.038). Accordingly, we assigned risk scores based on multivariable analysis-derived hazard ratios (HR) to the presence of 1 CV risk factor (HR=3.5; 1 point), >1 CV risk factors (HR=8.3; 2 points) and previous thrombosis (HR=2.0; 1 point). A final three-tiered prognostic model for thrombosis prediction was developed as low (score 0), intermediate (score 1 or 2) and high risk (score 3) (p<0.001). The hazard of thrombosis was 3.8% in low-risk group, 16.7% in the intermediate-risk group and 60% in the high-risk group (p<0.001). Patients with thrombotic complications during the follow-up had a significantly shorter survival (p=0.018). The new score based on CV risk factors and previous thrombotic events allows a better patient selection within prognostic-risk groups and improved identification of the high-risk patients for thrombosis.
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