Congenital Bleeding Disorders

Dabbagh

Tabibian

et al. 2020

Semin Thromb Hemost

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mentioning

confidence: 95%

Patients with Congenital Bleeding Disorders Appear to be Less Severely Affected by SARS-CoV-2: Is Inherited Hypocoagulability Overcoming Acquired Hypercoagulability of Coronavirus Disease 2019 (COVID-19)?

Dabbagh

Tabibian

et al. 2020

Semin Thromb Hemost

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“…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%

“…Bleeding disorders present a broad range, including rare and common coagulation factor (F) deficiencies, inherited platelet function disorders, fibrinolytic system disorders, and von Willebrand disease (VWD). 1 VWD is the most common congenital bleeding disorder, estimated to affect up to 1% of the population. Rare bleeding disorders are estimated to occur between 1 in 500,000 and 1 in 1 million.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Risk and Management of Intracerebral Hemorrhage in Patients with Bleeding Disorders

Farshi

Haeri

et al. 2022

Semin Thromb Hemost

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Intracerebral hemorrhage (ICH) is the most dreaded complication, and the main cause of death, in patients with congenital bleeding disorders. ICH can occur in all congenital bleeding disorders, ranging from mild, like some platelet function disorders, to severe disorders such as hemophilia A, which can cause catastrophic hemorrhage. While extremely rare in mild bleeding disorders, ICH is common in severe coagulation factor (F) XIII deficiency. ICH can be spontaneous or trauma-related. Spontaneous ICH occurs more often in adults, while trauma-related ICH is more prevalent in children. Risk factors that can affect the occurrence of ICH include the type of bleeding disorder and its severity, genotype and genetic polymorphisms, type of delivery, and sports and other activities. Patients with hemophilia A; afibrinogenemia; FXIII, FX, and FVII deficiencies; and type 3 von Willebrand disease are more susceptible than those with mild platelet function disorders, FV, FXI, combined FV–FVIII deficiencies, and type 1 von Willebrand disease. Generally, the more severe the disorder, the more likely the occurrence of ICH. Contact sports and activities can provoke ICH, while safe and noncontact sports present more benefit than danger. An important risk factor is stressful delivery, whether it is prolonged or by vacuum extraction. These should be avoided in patients with congenital bleeding disorders. Familiarity with all risk factors of ICH can help prevent occurrence of this diathesis and reduce related morbidity and mortality.

“…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%

Standardization of Prothrombin Time/International Normalized Ratio (PT/INR)

Favaloro

Bahraini

et al. 2020

Int J Lab Hematology

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The prothrombin time (PT) represents the most commonly used coagulation test in clinical laboratories. The PT is mathematically converted to the international normalized ratio (INR) for use in monitoring anticoagulant therapy with vitamin K antagonists such as warfarin in order to provide test results that are adjusted for thromboplastin and instrument used. The INR is created using two major PT ‘correction factors’, namely the mean normal PT (MNPT) and the international sensitivity index (ISI). Manufacturers of reagents and coagulometers have made some efforts to harmonizing INRs, for example, by tailoring reagents to specific coagulometers and provide associated ISI values. Thus, two types of ISIs may be generated, with one being a ‘general’ or ‘generic’ ISI and others being reagent/coagulometer‐specific ISI values. Although these play a crucial role in improving INR results between laboratories, these laboratories reported INR values are known to still differ, even when laboratories use the same thromboplastin reagent and coagulometer. Moreover, ISI values for a specific thromboplastin can vary among different models of coagulometers from a manufacturer using the same method for clot identification. All these factors can be sources of error for INR reporting, which in turn can significantly affect patient management. In this narrative review, we provide some guidance to appropriate ISI verification/validation, which may help decrease the variability in cross laboratory reporting of INRs.