Inherited platelet functional disorders: General principles and practical aspects of management

Lee, Adrienne; Poon, Man‐Chiu

doi:10.1016/j.transci.2018.07.010

Cited by 32 publications

(37 citation statements)

References 68 publications

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“…The prevalence of GT is estimated to be around one per million individuals in the general population [ 2 ]. However, highly consanguineous populations such as Iran, Canada, Newfoundland, and Jordan have an increased prevalence of approximately one per 200,000 individuals [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Medications should be reviewed with particular attention to nonsteroidal anti-inflammatory drugs as these therapies are frequent causes for acquired platelet functional defects. Initial laboratory screening tests should include a complete blood count, peripheral blood smear, plasma thromboplastin time (aPTT), thrombin time (PT), fibrinogen level, factor XIII screen, and von Willebrand disease studies, including VWF antigen, ristocetin cofactor activity, and factor VIII activity [ 5 ]. If the history and screening tests are in favor of an inherited platelet defect, additional testing such as light transmission aggregometry (LTA) and flow cytometry may be utilized for definitive diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with minor bleeding amenable to local control measures may be managed with pressure, ice packs, electrocauterization, and surgical repair [ 2 , 12 ]. Tranexamic acid which is an antifibrinolytic agent has also been utilized as a local hemostatic agent as in our case [ 5 ]. If the patients do not respond appropriately to local hemostatic techniques or if the condition worsens, systemic measures may be pursued.…”

Section: Discussionmentioning

confidence: 99%

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Glanzmann’s Thrombasthenia: How Listening to the Patient Is Sometimes the Simple Key to Good Medicine!

Jayakrishnan

Limonnik

Shah

et al. 2020

Case Reports in Medicine

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Introduction. Glanzmann’s thrombasthenia is a rare clotting disorder caused by impaired platelet function. Lack of awareness of the appropriate management of rare medical conditions may lead to patient dissatisfaction and potentially poor treatment outcome. Case Report. A 78-year-old male with a history of Glanzmann’s thrombasthenia was admitted to the trauma service following a fall in which he sustained a facial laceration as well as maxillary sinus and nasal fractures. He received DDAVP 20 mcg and tranexamic acid upon presentation to the emergency department (ED). In the ED, the patient requested administration of platelet transfusion but was refused due to a normal platelet count. During the course of his hospital stay, he complained of epistaxis and was noted to have a downtrending hemoglobin from 11.0 g/dl to 9.0 g/dl. The patient and his family were not comfortable when the discharge plan was finalized and demanded platelet transfusion (due to history of needing platelets in association with injuries or procedures in the past) was refused by the primary team as they continued to state that his platelet count is normal. On hospital day 3, hematology was consulted as the patient and his family were extremely angry and hematology recommended platelet transfusion. Further clinical information was not available as the patient was transferred to another facility per family request as they wanted to be at a center which had the patient’s primary hematologist. Discussion. A delay in specialist consultation resulted in patient dissatisfaction and extended the length of stay. Patients with rare medical conditions and potential for major complications should be managed aggressively with appropriate specialist consultation to promote patient satisfaction and improve the overall quality of care. This case shows that as physicians it our duty to listen to our patient’s concerns and involve them in the medical decision-making to provide optimal patient-centered care.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Glanzmann’s Thrombasthenia: How Listening to the Patient Is Sometimes the Simple Key to Good Medicine!

Jayakrishnan

Limonnik

Shah

et al. 2020

Case Reports in Medicine

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“…13 Scattered anecdotal reports and case series further suggested benefit of rFVIIa in patients with severe platelet function and other rare coagulation disorders. 14,15 The treatment was carried out before the recognition of blood group compatibility (there is however a 67% chance of compatibility by random selection of the UK population 2 ) and the era of anticoagulated blood transfusion. Activated clotting factors generated in the transfused un-anticoagulated blood were likely responsible for the effective haemostasis whether in haemophilia or other bleeding disorders.…”

Section: Samuel Armstrong Lane's First Successful Treatment Of Haemopmentioning

confidence: 99%

Samuel Armstrong Lane's first successful treatment of haemophilia with blood transfusion in 1840: Could this also be the first successful bypassing therapy?

Poon

Card

2018

Haemophilia

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“…This activates platelets and induces secretion of platelet granules, which recruits more platelets to form a solid haemostatic plug. Simultaneously, exposed tissue factors will activate the clotting cascade, producing fibrin that strengthens the platelet plug and forming a clot (secondary haemostasis) (Broos, Feys, De Meyer, Vanhoorelbeke, & Deckmyn, 2011;Lee & Poon, 2018).…”

mentioning

confidence: 99%

World Workshop on Oral Medicine VII: Platelet count and platelet transfusion for invasive dental procedures in thrombocytopenic patients: A systematic review

et al. 2019

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Objectives To evaluate the evidence for a 50,000/μl platelet count threshold for platelet transfusion for invasive dental procedures in thrombocytopenic patients. Subjects and Methods We searched in MEDLINE/PubMed, EMBASE, the Cochrane Library (Wiley) and Scopus from 1960 through April 2018 for studies on patients with quantitative platelet disorders not related to medical co‐morbidities or medications and undergoing invasive dental procedures. Two reviewers conducted assessments independently. Results We found a total of 176 non‐duplicate articles, of which 9 cohort studies met our inclusion criteria. The incidence of postoperative bleeding in thrombocytopenic patients was low (4.9%), and we found no difference in bleeding incidence between patients who had platelet transfusion and those who did not. There was no difference in the mean platelet count for patients with and without bleeding. Different modalities are now available to prevent and control bleeding, which may reduce the need for platelet transfusion. Conclusions There is no evidence to support the long‐standing dogma of a need for a platelet count ≥ 50,000/μl for safe invasive dental procedures. Platelet transfusion effectiveness for haemostasis support could not be determined based on available data. Local measures and antifibrinolytics are the mainstay for the prevention and management of bleeding.

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Inherited platelet functional disorders: General principles and practical aspects of management

Cited by 32 publications

References 68 publications

Glanzmann’s Thrombasthenia: How Listening to the Patient Is Sometimes the Simple Key to Good Medicine!

Glanzmann’s Thrombasthenia: How Listening to the Patient Is Sometimes the Simple Key to Good Medicine!

Samuel Armstrong Lane's first successful treatment of haemophilia with blood transfusion in 1840: Could this also be the first successful bypassing therapy?

World Workshop on Oral Medicine VII: Platelet count and platelet transfusion for invasive dental procedures in thrombocytopenic patients: A systematic review

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