Discrepant ratios of arterial vs. venous thrombosis in hemophilias A and B as compared to FVII deficiency

Background: The occurrence of a thrombotic event in congenital bleeding disorders has drawn considerable attention in recent years. Both patients with hemophilia and patients with von Willebrand disease and even those with rare coagulation disorders have been shown to present occasional thrombotic events. Little is known on the comparative prevalence of arterial vs. venous thrombosis in these patients. Objectives: The purpose of the present investigation was to evaluate the prevalence of arterial vs. venous occlusions in hemophilia A and B vs. FVII de\ufb01ciency. Methods: A time unlimited search of the literature was carried out using pertinent key words. Arterial or venous occlusions had to be proven by objective methods. Results: Eighty-\ufb01ve patients with hemophilia A or B have been reported to have had an arterial occlusion vs. six cases of FVII de\ufb01ciency. On the contrary, 34 patients with hemophilia A or B and 32 cases with FVII de\ufb01ciency have been reported to have presented with a venous thrombosis. The ratios of arterial vs. venous thrombosis are 3.72, 1.13, and 2.50 for hemophilia A, hemophilia B, and hemophilia A + B combined, respectively, and 0.19 for FVII de\ufb01ciency. Conclusions: Hemophilia A and hemophilia B do not protect from arterial occlusions (mainly acute coronary syndromes), whereas they assure some protection from venous thrombosis. The opposite seems true for FVII de\ufb01ciency. The potential signi\ufb01cance of this discrepancy is discusse

Correlation between ADAMTS13 activity and neurological impairment in acute thrombotic microangiopathy patients

Differential diagnosis between thrombotic thrombocytopenic purpura (TTP) and other thrombotic microangiopathies (TMA) is usually difficult because of frequently overlapping clinical presentations. Severely depressed ADAMTS13 activity (<10\ua0%) seems distinctive for TTP because of its pathogenetic role. However a long debate exists in the literature about its sensibility and specificity. Our aim was to search for clinical differences between TMA patients referred to our laboratory, comparing them for protease activity\ua0<10 versus\ua0 6510\ua0%. ADAMTS13 activity\ua0 6510\ua0% patients (n\ua0=\ua073) showed a higher prevalence of drug- (p\ua0=\ua00.005) and cancer-associated (p\ua0<\ua00.001) TMA. Mean platelet count and renal dysfunction prevalence was lower (p\ua0<\ua00.001), while neurological impairment was more frequent (p\ua0=\ua00.001) in the\ua0<10\ua0% ADAMTS13 activity group (n\ua0=\ua0109), confirming previous literature findings. When taken neurological manifestations singularly, epilepsy (p\ua0=\ua00.04), focal motor deficit (p\ua0<\ua00.001) and cranial nerve palsy (p\ua0=\ua00.007) were more frequent in the\ua0<10\ua0% activity group. In our case series, a\ua0<10\ua0% ADAMTS13 activity depicts a group of patients with clinical features similar to TTP patients. Focal motor impairment or epileptic manifestations could further address toward a TTP diagnosis. Studies about treatment efficacy and follow-up are advised to determine whether laboratory findings can guide therapeutic decisions

Weight loss reduces anti-ADAMTS13 autoantibodies and improves inflammatory and coagulative parameters in obese patients

Obese patients have been described at increased risk of thrombotic thrombocytopenic purpura, a disease caused by anti-ADAMTS13 autoantibodies. ADAMTS13 has a structure homology with the adipokine thrombospondin-1. We previously demonstrated an increased presence of anti-ADAMTS13 antibodies in obese patients. We aimed to study the changes induced by weight loss after bariatric surgery on some inflammatory and coagulative parameters and their link with anti-ADAMTS13 autoantibodies. We studied 100 obese patients before and after weight loss induced by bariatric surgery and 79 lean volunteers as controls. We measured anthropometric, metabolic and inflammatory parameters, thrombospondin-1, ADAMTS13 activity, anti-ADAMTS13 autoantibodies, Von Willebrand factor. At baseline, 13\u2009% of patients was positive for anti-ADAMTS13 autoantibodies, while all controls were negative. Thrombospondin-1 levels were higher in obese subjects with than without antibodies, with a positive correlation between the two parameters. In multiple logistic regression analysis only thrombospondin-1 levels predicted positivity for anti-ADAMTS13 antibodies. After weight loss both anti-ADAMTS13 antibodies and thrombospondin-1 reduced significantly. Weight loss in obesity improves the inflammatory and coagulative profile, and in particular anti-ADAMTS13 autoantibodies, ADAMTS13 activity and thrombospondin-1

Using high sensitivity cardiac troponin values in patients with SARS-CoV-2 infection (COVID-19): The Padova experience

BACKGROUND: The spectrum of Coronavirus Disease 2019 (COVID-19) is broad and thus early appropriate risk stratification can be helpful. Our objectives were to define the frequency of myocardial injury using high-sensitivity cardiac troponin I (hs-cTnI) and to understand how to use its prognostic abilities.METHODS: Retrospective study of patients with COVID-19 presenting to an Emergency Department (ED) in Italy in 2020. Hs-cTnI was sampled based on clinical judgment. Myocardial injury was defined as values above the sex-specific 99th percentile upper reference limits (URLs). Most data is from the initial hospital value.RESULTS: 426 unique patients were included. Hs-cTnI was measured in 313 (73.5%) patients; 85 (27.2%) had myocardial injury at baseline. Patients with myocardial injury had higher mortality during hospitalization (hazard ratio=9 [95% confidence interval (CI) 4.55-17.79], p13 [OR (95% CI): 0.48 (0.08, 2.65), p=0.40]. Initial hs-cTnI<5ng/L identified 33% of patients that were at low risk with 97.8% sensitivity (95% CI 88.7, 99.6) and 99.2% negative predictive value. Type 1 myocardial infarction (MI) and type 2 MI were infrequent.CONCLUSIONS: hs-cTnI at baseline is a significant predictor of mortality in COVID-19 patients. A value<5ng/L identified patients at low risk

Discrepant ratios of arterial versus venous thrombosis in hemophilia A as compared with hemophilia B

The occurrence of thrombosis in patients with congenital bleeding disorders represents an exceptional event. Hemophilia A and hemophilia B patients have been showed to present both arterial and venous thrombosis (85 cases of arterial thrombosis and 34 cases of venous thrombosis). The great majority of arterial thrombosis are myocardial infarction or other acute coronary syndromes, whereas the majority of venous thrombosis are deep vein thrombosis and/or pulmonary embolisms. However there are discrepancies in the proportion of arterial and venous thrombosis seen in hemophilia A versus hemophilia B. The ratio of arterial versus venous thrombosis in hemophilia A is 3.72 whereas that for hemophilia B is 1.12. This indicates that arterial thrombosis is more frequent in hemophilia A as compared to hemophilia B and the opposite is true for venous thrombosis. The potential signi\ufb01cance of this discrepancy is discussed