Guideline for the diagnosis and management of the rare coagulation disorders

Mumford, Andrew D; Ackroyd, Sam; Alikhan, Raza; Bowles, Louise; Chowdary, Pratima; Grainger, John D.; Mainwaring, Jason; Mathias, Mary; O’Connell, Niamh M

doi:10.1111/bjh.13058

Cited by 262 publications

(148 citation statements)

References 200 publications

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“…Most hemostatic abnormalities were mild and would not prompt treatment in clinical practice 9, 23. Moreover, patients reporting ≥1 bleeding symptoms on the anaesthesiology questionnaire did not have more abnormalities than those who did not report bleeding symptoms, nor were these more severe.…”

Section: Discussionmentioning

confidence: 99%

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Preoperative screening for bleeding disorders: A comprehensive laboratory assessment of clinical practice

Vries

Meijden

Kuiper

et al. 2018

Research and Practice in Thrombosis and Haemostasis

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Essentials Patients with bleeding disorders are at risk of operative bleeding, but screening for these disorders is challenging.Patients with and without bleeding symptoms on a guideline‐based screening questionnaire were included and hemostatically phenotyped.The questionnaire could not differentiate between patients with and without hemostatic abnormalities.The discriminative power of the PT, aPTT, TT, Euglobulin lysis time, PFA, and the ISTH‐BAT was also limited. BackgroundPatients with mild bleeding disorders are at risk of perioperative bleeding, but screening for these disorders remains challenging.ObjectivesWe aimed to assess the prevalence of hemostatic abnormalities in patients with and without reported bleeding symptoms on a preoperative questionnaire, consisting of guideline‐proposed questions, and appraised the diagnostic value of several screening modalities for the identification of patients with hemostatic abnormalities.MethodsIn this observational study, 240 patients with and 95 patients without bleeding symptoms on the preoperative questionnaire were included. Patients with known bleeding disorders, antithrombotic drugs, thrombocytopenia, and anemia were excluded. Preoperatively, all patients underwent elaborate hemostatic testing. Hemostatic abnormalities were defined as coagulation, vWF, or fibrinolysis factor levels below reference range and platelet function defects. Screening modalities included the ISTH Bleeding Assessment Tool (ISTH‐BAT), PT, aPTT, TT, Euglobulin Lysis Time (ELT), and Platelet Function Analyser (PFA).ResultsIn 21 of 240 (8.8%) patients reporting bleeding symptoms, hemostatic abnormalities were found, including 7 reduced coagulation factor levels, 10 platelet function abnormalities, and 4 reduced vWF levels. In comparison, 10 of 95 (10.5%) patients not reporting bleeding symptoms had abnormalities. The ISTH‐BAT could not identify patients with abnormalities, while PT, aPTT, TT, ELT, and PFA had high specificity but low sensitivity to detect abnormalities.ConclusionsThe prevalence of hemostatic abnormalities in both patients with and without reported bleeding symptoms was 9%‐10%. This suggests that the guideline‐based questionnaire cannot differentiate between patients with and without abnormalities, while the discriminative power of the screening modalities is also limited.

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Section: Discussionmentioning

confidence: 99%

“…The execution of coagulation tests such as the PT or aPTT is not favored, as these tests were proven insensitive to mild deficiencies 7, 8, 9. In spite of this, a recent survey revealed that these tests are used strikingly often by anesthesiologists 10.…”

Section: Introductionmentioning

confidence: 99%

Preoperative screening for bleeding disorders: A comprehensive laboratory assessment of clinical practice

Vries

Meijden

Kuiper

et al. 2018

Research and Practice in Thrombosis and Haemostasis

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“…La presencia de anticuerpos anti protrombina o factor II al formar complejos inmunes, son eliminados rápidamente de la sangre produciendo una hipoprotrombinemia generalmente severa. Se esperaría que los niveles de factor II se encuentren en rango muy bajos al diagnóstico (media: 11%; IC: 1%-40%) (4,(6)(7)(8).…”

Section: Dra Carolina Tokumuraunclassified

Caso clínico 03-2017. Mujer de 14 años con gingivorragia, ginecorragia y artralgias

Tokumura¹,

Guevara²,

Gonzales³

2017

Rev Med Hered

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“…Significant or prolonged bleeding after injury or surgery with or without exsanguinations requiring blood product therapy, others includes obstetric haemorrhage, bleeding from dental procedures, bleeding from circumcision., bleeding during tumor surgery, other massive surgical haemorrhage, neurosurgical bleeding in the cranium, bleeding into cavities like joints, pleural space, abdomen, and muscular hematomas. [10][11][12] There is a battery of coagulation screening / tests to guide patient management in the Perioperative period. Whenever findings in the history, such as comorbidities, previous abnormal bleeding, a detailed family history, current medications (antiplatelet, oral anticoagulants, fibrinolytic), and physical examination suggests an increase risk of significant bleeding, then it is more cost effective to carry out routine screening tests of coagulation (especially before invasive procedures), such as Full blood count, platelet count, prothrombin time (PT) / activated partial thromboplastin time (aPTT).…”

Section: Pathophysiologymentioning

confidence: 99%

“…Vitamin k in doses of 0.1-0.5mg/kg has been used to treat hemorrhagic disease of newborn. [13][14][15] Treatment of identified specific factor deficiency with specific recombinant factors concentrate, such as FXII A-subunit and FVIIa, or virally inactivated plasma derived factor concentrates such as Fibrinogen, FVII(novo-seven), FX, FXI, and FXIII(novo-thirteen). PCC is a four factor, plasma derived concentrate (FII, FVII, FIX & FX), for the treatment of prothrombin deficiency, vitamin k dependent factor deficiency, and for FX, FVII.…”

Section: Pathophysiologymentioning

confidence: 99%

Perioperative Coagulopathy and the Clinical Implications

Orilonise¹

2016

AIMDR

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Perioperative bleeding as conceived in this review refers to the clinical scenario whereby there is impairment of blood coagulation leading to prolonged or excessive bleeding, accompanying trauma or surgical exposure. Aetiology of bleeding disorders could be classified under the following subheadings: vascular disorders, Thrombocytopenia, Platelet function defects, Coagulation factors disorder(s). Primary haemostasis is initiated or triggered by the release of tissue factor at the site of injury leading to generation of small quantity thrombin, sufficient to activate platelets leading to the formation of prothrombin complex. The ability of FXa to activate FVII creates a link between the extrinsic and intrinsic coagulation pathways; leading to the generation of sufficient thrombin to convert fibrinogen to fibrin which organizes platelet plug to indissoluble clots (Secondary haemostasis) or thrombus formation. A balance is required between haemostasis, thrombosis and fibrinolysis to prevent perioperative coagulopathy. Whenever positive findings in the history and physical examination suggests an increased risk of significant bleeding, then it is more cost effective to carry out routine screening tests of coagulation. Specific coagulation tests used routinely has longer turnaround times and is not appropriate in the perioperative setting. Current recommendations for the prevention of massive bleeding in the perioperative setting; requires communication of appropriate treatment plans in a multidisciplinary setting, intraoperative monitoring, the treatment of underlying disorder, and replacement therapy with blood products. As point-of-care diagnostics becomes available in emergency areas, timely targeted intervention for haemorrhage control will result in better patient outcomes and reduced demand for blood products.

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Guideline for the diagnosis and management of the rare coagulation disorders

Cited by 262 publications

References 200 publications

Preoperative screening for bleeding disorders: A comprehensive laboratory assessment of clinical practice

Preoperative screening for bleeding disorders: A comprehensive laboratory assessment of clinical practice

Caso clínico 03-2017. Mujer de 14 años con gingivorragia, ginecorragia y artralgias

Perioperative Coagulopathy and the Clinical Implications

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