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Haematological Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
These tests should be performed if there is any evidence of a bleeding disorder. Their use in patients without a bleeding history to ‘screen’ for disease is controversial and can lead to misleading results.
Applied Surgical Anatomy
Published in Tjun Tang, Elizabeth O'Riordan, Stewart Walsh, Cracking the Intercollegiate General Surgery FRCS Viva, 2020
Vishal G Shelat, Andrew Clayton Lee, Julian Wong, Karen Randhawa, CJ Shukla, Choon Sheong Seow, Tjun Tang
What is the source of bleeding?GDA pseudoaneurysm is a common source of bleeding following pancreatic resections.This usually presents with warning or herald signs of haemorrhagic drain output and then overtly presents with significant bleed, which leads to haemodynamic instability.CT angiogram is the investigation of choice with embolisation as the therapeutic option in patients with a blush.Intra-operatively, a surgeon could take some precautions to avoid GDA pseudoaneurysm: Leave behind a longer stumpCover the stump with tissue, e.g., falciform ligament; omentumCover the stump with topical haemostatic productsMark the stump with metal clips to facilitate detection by imagingPlace drain in the proximity of pancreatic anastomosis
Bleeding in acute coronary syndrome
Published in K Sarat Chandra, AJ Swamy, Acute Coronary Syndromes, 2020
Bleeding is often merely regarded as an unpleasant event, which increases the length of hospital stay and costs without adversely affecting survival. Consequences of bleeding include hypotension, anaemia and reduction in oxygen delivery. Furthermore, severe bleeding increases the risk of acute myocardial infarction, stroke and the need for urgent myocardial revascularisation [17,20]. In post-PCI patients it also requires stoppage of DAPT leading to a high risk of stent thrombosis.
Testing strategies used in the diagnosis of rare inherited bleeding disorders
Published in Expert Review of Hematology, 2023
Patients with a high bleeding score but normal results of hemostatic testing may have collagen vascular bleeding disorder such as Ehlers-Danlos Syndrome (EDS). Major clinical manifestations of EDS variants include joint hypermobility, skin fragility, and hyperextensibility. The Beighton screening tool assesses for joint hypermobility (Table 3), seen in selected variants of EDS and assigns a score. This score provides a likelihood of joint hypermobility [12]; additional clinical findings in EDS variants with bruising as a prominent symptom are shown in Table 4. More detailed description of EDS variants is described in recent publications [13]. Although EDS variants were felt to be rare and are not included in the definition of RBD, a recent study estimated a prevalence of approximately 1 in 500 in the general population [14]. Follow-up testing for EDS, if indicated, consists of molecular genetic testing for genes implicated in each subtype of EDS listed in (Table 4). The modes of inheritance and genes implicated in the pathogenesis of each subtype vary. It should be noted that patients with EDS may also have co-existing hemostatic abnormalities [15]. Most of these variants have a relatively benign clinical course except for vascular type (formerly type IV), which has a propensity for life-threatening complications, such as arterial rupture. Finally, despite exhaustive testing, a defined group of patients with an evident bleeding tendency, but no diagnostic laboratory findings are categorized as bleeding disorders of unknown cause [16].
New αIIbβ3 variants in 28 Turkish Glanzmann patients; structural hypothesis for complex activation by residues variations in I-EGF domains
Published in Platelets, 2022
M Y Koker, N Sarper, C Albayrak, B Zulfikar, E Zengin, B Saraymen, D Albayrak, B Koc, H Avcilar, M Karakükcü, C Chenet, F Bianchi, A G de Brevern, R Petermann, V Jallu
Patients presenting with moderate to severe mucosal bleeding symptoms and purpura were diagnosed in four Pediatric Hematology Centers, mostly located in the North-West of Turkey. Main symptoms were severe nose and gum bleeding, and menorrhagia. Detailed symptoms are reported in Table S1 (Supporting information 1). Platelet counts and morphology on peripheral blood smears were normal, activated partial thromboplastin time and prothrombin time were not prolonged. Closure time measured by platelet function analyzer 100 (PFA-100) was prolonged. Plasma fibrinogen and von Willebrand factor levels were within normal limits. Platelet aggregation by lumiaggregometer showed impaired aggregation with physiologic agonists (ADP, collagen, and arachidonic acid) but normal agglutination with Ristocetin. A first routine flow cytometry analysis revealed an impaired expression of αIIbβ3 in patients’ platelets confirming the GT diagnosis (not shown). Blood samples from 28 patients described herein and their close relatives (parents, siblings) belonging to 20 unrelated families were used in further phenotypic and genetic studies. None of these patients had been previously studied for molecular gene defects.
Primary immune thrombocytopenia in adults: Belgian recommendations for diagnosis and treatment anno 2021 made by the Belgian Hematology Society
Published in Acta Clinica Belgica, 2022
A. Janssens, D. Selleslag, J. Depaus, Y. Beguin, C. Lambert
Many patients remain asymptomatic with the diagnosis of ITP made only after a routine blood test. Others may have bleeding symptoms ranging from skin bleeding (petechiae, purpura, bruises) (dry purpura) to mucosal bleeding (gingival bleeding, mouth blisters, epistaxis, blood in stool or urine, heavy menstrual bleeding) or intracranial bleeding (wet purpura) appearing spontaneously or after trauma. Bleeding symptoms have mostly been reported in patients with a platelet count of less than 30 x 109/L while life-threatening bleedings are rare in patients with platelets higher than 10 x 109/L. Other factors influencing the bleeding risk are age, comorbidities, lifestyle, need for invasive procedures, need of treatment with anticoagulant or antiplatelet agents, …