Congenital Bleeding Disorders 2018
DOI: 10.1007/978-3-319-76723-9_2
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Congenital Bleeding Disorders

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Cited by 7 publications
(17 citation statements)
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“…It is postulated that subsequent release of a large amount of von Willebrand factor swamps ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), resulting in platelet aggregates and pulmonary thrombosis, 15 a phenomenon that is less likely in some CBDs, especially type 3 VWD and Bernard-Soulier's syndrome, thus making them less susceptible to any such related fatal event. 16 In conclusion, it appears that a severe hypocoagulability state may be protective against COVID-19 hypercoagulability-related adverse effects, an important consideration otherwise making anticoagulation mandatory for all patients hospitalized with COVID-19. Some authors suggest mild anticoagulant therapy, whereas others prefer more aggres-sive therapeutic strategies.…”
mentioning
confidence: 95%
“…It is postulated that subsequent release of a large amount of von Willebrand factor swamps ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), resulting in platelet aggregates and pulmonary thrombosis, 15 a phenomenon that is less likely in some CBDs, especially type 3 VWD and Bernard-Soulier's syndrome, thus making them less susceptible to any such related fatal event. 16 In conclusion, it appears that a severe hypocoagulability state may be protective against COVID-19 hypercoagulability-related adverse effects, an important consideration otherwise making anticoagulation mandatory for all patients hospitalized with COVID-19. Some authors suggest mild anticoagulant therapy, whereas others prefer more aggres-sive therapeutic strategies.…”
mentioning
confidence: 95%
“…Iso-antibodies formation is less commonly reported in gray platelet syndrome (∼1%) and Bernard–Soulier syndrome (∼1%). 1 The majority of Bernard–Soulier syndrome cases do not require regular treatment. Alloimmunization, as with Glanzmann thrombasthenia patients, remains the main obstacle of platelet transfusion.…”
Section: Management Of Ich In Congenital Bleeding Disordersmentioning
confidence: 99%
“…5 Besides, ICH most frequently occurs in patients with afibrinogenemia, prothrombin deficiency, FVII, FVIII, FIX, FX deficiencies, Glanzmann thrombasthenia, and type 3 VWD. 1 2 4 Mild bleeding tendencies, such as FXI deficiency and some fibrinolytic system disorders, may occasionally cause ICH. While ICH is the main cause of morbidity and mortality in most patients with bleeding disorders, an appropriate therapeutic strategy can significantly decrease its occurrence and improve the quality of life for those affected.…”
mentioning
confidence: 99%
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“…Recent studies indicate that the TF pathway reflects the major driver of in vivo coagulation 2,5‐7 . A prolonged PT might be due to one or more coagulation factor defects in the TF or common pathways, or might indicate the presence of inhibitors against these factors 2,8 .…”
Section: Introductionmentioning
confidence: 99%