Inherited platelet diseases with normal platelet count: phenotypes, genotypes and diagnostic strategy

P Nurden, S Stritt, R Favier, AT Nurden - Haematologica, 2020 - pmc.ncbi.nlm.nih.gov
Inherited platelet disorders resulting from platelet function defects and a normal platelet
count cause a moderate or severe bleeding diathesis. Since the description of Glanzmann …

How we treat the platelet glycoprotein defects; Glanzmann thrombasthenia and Bernard Soulier syndrome in children and adults

JD Grainger, J Thachil, AM Will - British journal of haematology, 2018 - Wiley Online Library
The inherited platelet glycoprotein deficiencies, Glanzmann thrombasthenia (GT) and
Bernard Soulier syndrome (BSS) are rare but important long‐term bleeding disorders. Once …

[HTML][HTML] Bleeding risk of surgery and its prevention in patients with inherited platelet disorders

S Orsini, P Noris, L Bury, PG Heller, C Santoro… - …, 2017 - ncbi.nlm.nih.gov
Excessive bleeding at surgery is a feared complication in patients with inherited platelet
disorders. However, very few studies have evaluated the frequency of surgical bleeding in …

Platelet δ-storage pool disease: an update

A Dupuis, JC Bordet, A Eckly, C Gachet - Journal of clinical medicine, 2020 - mdpi.com
Platelet dense-granules are small organelles specific to the platelet lineage that contain
small molecules (calcium, adenyl nucleotides, serotonin) and are essential for the activation …

[HTML][HTML] Consensus report on flow cytometry for platelet function testing in thrombocytopenic patients: communication from the SSC of the ISTH

G Jourdi, S Ramström, R Sharma, T Bakchoul… - Journal of Thrombosis …, 2023 - Elsevier
Background Platelet count alone does not reliably predict bleeding risk, suggesting platelet
function is important to monitor in patients with thrombocytopenia. There is still an unmet …

The ISTH bleeding assessment tool as predictor of bleeding events in inherited platelet disorders: Communication from the ISTH SSC Subcommittee on Platelet …

P Gresele, E Falcinelli, L Bury, A Pecci… - … of Thrombosis and …, 2021 - Wiley Online Library
Abstract Background The ISTH Bleeding Assessment Tool (ISTH‐BAT) has been validated
for clinical screening of suspected von Willebrand disease (VWD) and for bleeding …

How I manage pregnancy in women with Glanzmann thrombasthenia

M Fiore, L Sentilhes, R d'Oiron - Blood, The Journal of the …, 2022 - ashpublications.org
Glanzmann thrombasthenia (GT) is a rare inherited platelet function disorder caused by a
quantitative and/or qualitative defect of the αIIbβ3 integrin. Pregnancy and delivery are …

[HTML][HTML] Validation of immunofluorescence analysis of blood smears in patients with inherited platelet disorders

C Zaninetti, E Leinøe, ML Lozano, M Rossing… - Journal of Thrombosis …, 2023 - Elsevier
Abstract Background Inherited platelet disorders (IPDs) are rare diseases characterized by
reduced blood platelet counts and/or impaired platelet function. Recognizing IPDs is …

The origin of platelets enabled the evolution of eutherian placentation

JF Martin, GP Wagner - Biology Letters, 2019 - royalsocietypublishing.org
Invasive placentation with extended pregnancy is a shared derived characteristic unique to
eutherian mammals that possess a highly effective system of haemostasis, platelets. These …

[HTML][HTML] Platelet function testing at low platelet counts: When can you trust your analysis?

N Boknäs, AS Macwan, AL Södergren… - Research and practice in …, 2019 - Elsevier
Background Although flow cytometry is often brought forward as a preferable method in the
setting of thrombocytopenia, the relative effects of low sample counts on results from flow …