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Variable Clinical Presentation of Congenital Thrombotic Thrombocytopenic Purpura in a Large Cohort of Patients Carrying a New Mutation in the CUB1 Domain of ADAMTS13 Gene
Conclusion:To the best of our knowledge, this is one of the largest congenital TTP cohorts described in the literature. This cohort is unique due to the fact that all members carry the same, previously unreported, ADAMTS13 gene mutation in the CUB1 domain of the gene. Our findings support an assumption that environmental and hereditary modifiers may influence disease course. Further research of the involved families may enable us to expand the understanding of the pathophysiology and develop better treatment options for this understudied rare condition.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Pikovsky, O., Arafat, M., Ovadia, H., Sharoni, Y., Al-Athamen, K., Kanengisser-Pines, B., Keren-Politansky, A., Levi, I., Parvari, R., Rabinovich, A. Tags: 311. Disorders of Platelet Number or Function: Poster I Source Type: research

Direct Oral Anticoagulants and Mortality in Atrial Fibrillation
ConclusionsIn this cohort of newly diagnosed NVAF patients treated in routine clinical practice, DOAC therapy was associated with a lower risk for death compared to no oral anticoagulation. Our findings provide further evidence for the importance of DOAC therapy in NVAF patients.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Ellis, M., Ruslan, S., Ariel, H., Greenberg-Dotan, S., Battat, E., Hamburger-Avnery, O., Greenberg, D., Ronen, A. Tags: 332. Antithrombotic Therapy Source Type: research

Middle Cerebral Artery Velocities Are Inversely Related to Hemoglobin Levels and Acutely Drop in Response to RBC Transfusion: Implications for Stroke Screening in SCD
DiscussionThese data show a striking relationship between anemia and TCD velocity, and that RBC transfusion can alter MCA velocity. Even an 11% increase in hemoglobin such as that seen in the low-responder in Figure 3 could change a patient classification from high-risk to conditional TCD. These data also show the significant sensitivity of MCA velocity to the depth of measurement (Figure 2 & 3). The investigators in the STOP study went to extreme ends to standardize these measurements. Nonetheless, TCD measures may not be done with such rigor in clinical settings. It is clear that standard TCD screening has been of tr...
Source: Blood - November 21, 2018 Category: Hematology Authors: Perumbeti, A., Carreras, O., Detterich, J. A., Shah, P., Sunwoo, J., Khoo, M., Wood, J. C., Coates, T. D. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research