Platelet concentrate effects on thromboelastography

This study evaluated platelet effects on thromboelastography to determine how morphologically abnormal platelets affected native whole blood analysis. Prospective, controlled comparison. Tertiary care university hospital. Volunteer cardiac surgery patients. Fresh platelets were obtained from volunteers and were either treated normally or cryodisrupted with liquid nitrogen. Fresh platelets, liquid nitrogen-treated platelets, or an equivalent quantity of the patient's blood were added to whole blood samples obtained from cardiac surgery patients before heparinization. Thromboelastographic parameters sensitive to platelet effects were measured in each of the three groups.

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Maximum amplitude and a-angle significantly increased in the two groups receiving added platelets. There were no differences between the fresh platelet and the liquid nitrogen-treated platelet groups (Student's paired t-test). The R-time decreased significantly in both platelet-treated groups compared with the group that did not receive additional platelets. Viscoelastic changes in whole blood coagulation after the addition of platelet concentrates are not dependent on morphologically intact or functionally normal platelets. This in vitro study predicts that transfusion of poorly preserved platelet concentrates as well as fresh platelets would increase clot strength on thromboelastography if the recipient's blood were tested immediately after administration.     

Demographics and patient characteristics of 1209 patients with Gaucher disease: Descriptive analysis from the Gaucher Outcome Survey (GOS)

The Gaucher Outcome Survey (GOS) is an international Gaucher disease (GD) registry established in 2010 for patients with a confirmed GD diagnosis, regardless of GD type or treatment status, designed to evaluate the safety and long‐term effectiveness of velaglucerase alfa and other GD‐related treatments. As of February 25, 2017, 1209 patients had enrolled, the majority from Israel (44.3%) and the US (31.4%). Median age at GOS entry was 40.4 years, 44.1% were male, and 13.3% had undergone a total splenectomy. Most patients had type 1 GD (91.5%) and were of Ashkenazi Jewish ethnicity (55.8%). N370S/N370S was the most prevalent genotype, accounting for 44.2% of genotype‐confirmed individuals (n = 847); however, there was considerable variation between countries. A total of 887 (73.4%) patients had received ≥1 GD‐specific treatment at any time, most commonly imiglucerase (n = 587), velaglucerase alfa (n = 507), and alglucerase (n = 102). Hematological and visceral findings at the time of GOS entry were close to normal for most patients, probably a result of previous treatment; however, spleen volume of patients in Israel was almost double that of patients elsewhere (7.2 multiples of normal [MN] vs. 2.7, 2.9 and 4.9 MN in the US, UK and rest of world), which may be explained by a greater disease severity in this cohort. This analysis aimed to provide an overview of GOS and present baseline demographic and disease characteristics of participating patients to help improve the understanding of the natural history of GD and inform the overall management of patients with the disease.