The elusive and heterogeneous pattern of type 2M von Willebrand disease: A diagnostic challenge

Casonato, Alessandra; Galletta, Eva; Daidone, Viviana

doi:10.1111/ejh.13159

Cited by 3 publications

(1 citation statement)

References 34 publications

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“…PFA-100 is extensively studied in patients with (suspected) VWD. 17 18 19 21 22 23 24 26 27 28 29 30 31 32 33 34 35 36 37 43 51 52 53 56 57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 Favaloro et al reported an overall sensitivity of 83.2% and 91.5% of the ADP and EPI cartridge respectively in a review in 2008. 79 A current analysis including all articles published before and after 2008 yielded similar test performance outcomes, as shown in Fig.…”

Section: Results Of the Point-of-care Tests In Patients With Heredita...mentioning

confidence: 99%

Point-of-Care Testing in Patients with Hereditary Disorders of Primary Hemostasis: A Narrative Review

Bavinck,

Heerde,

Schols

2024

Semin Thromb Hemost

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Inherited disorders of primary hemostasis, such as von Willebrand disease and congenital platelet disorders, can cause extensive, typically mucocutaneous bleeding. Assays to diagnose and monitor these disorders, such as von Willebrand factor activity assays and light transmission aggregometry, are performed in specialized hemostasis laboratories but are commonly not available in local hospitals. Due to the complexity and relative scarcity of these conventional assays, point-of-care tests (POCT) might be an attractive alternative in patients with hereditary bleeding disorders. POCTs, such as thromboelastography, are increasingly used to assess hemostasis in patients with acquired hemostatic defects, aiding clinical decision-making in critical situations, such as during surgery or childbirth. In comparison, the use of these assays in patients with hereditary hemostasis defects remains relatively unexplored. This review aims to give an overview of point-of-care hemostasis tests in patients with hereditary disorders of primary hemostasis. A summary of the literature reporting on the performance of currently available and experimental POCTs in these disorders is given, and the potential utility of the assays in various use scenarios is discussed. Altogether, the studies included in this review reveal that several POCTs are capable of identifying and monitoring severe defects in the primary hemostasis, while a POCT that can reliably detect milder defects of primary hemostasis is currently lacking. A better understanding of the strengths and limitations of POCTs in assessing hereditary defects of primary hemostasis is needed, after which these tests may become available for clinical practice, potentially targeting a large group of patients with milder defects of primary hemostasis.

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