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JPMA. the Journal of the Pakistan... Sep 2021Pseudo thrombocytopenia is the estimation of low platelet counts by a Haematology analyzer despite of shortage in platelets. EDTA-induced pseudo thrombocytopenia,...
Pseudo thrombocytopenia is the estimation of low platelet counts by a Haematology analyzer despite of shortage in platelets. EDTA-induced pseudo thrombocytopenia, commonly seen in clinical practice, occurs mainly due to the anti-platelet antibodies. Pseudo thrombocytopenia is seen in normal healthy individuals and other disorders like cardiovascular, liver, autoimmune diseases and malignancy. We are presenting a case of multi-coagulant resistant dependent thrombocytopenia. The purpose of this letter is to review approaches to pseudo thrombocytopenia. The case has coagulant resistant dependent thrombocytopenia in association with Anasarca and was a known case of cardiomyopathy with severely dilated left atrium, left ventricle and right atrium.
Topics: Autoimmune Diseases; Blood Platelets; Edetic Acid; Humans; Platelet Count; Thrombocytopenia
PubMed: 34580523
DOI: 10.47391/JPMA.03-390 -
Journal of Thrombosis and Haemostasis :... Jun 2020Platelets are most important in providing cellular hemostasis but also take part in inflammation and immune processes. Increased platelet size has been regarded as a... (Review)
Review
Platelets are most important in providing cellular hemostasis but also take part in inflammation and immune processes. Increased platelet size has been regarded as a feature describing a young and more reactive subpopulation until studies were published which questioned this concept. Moreover, changes of platelet size given by the mean platelet volume (MPV) were described for immune thrombocytopenia, cardiovascular disease, atherosclerosis, venous thromboembolism, chronic lung disease, sepsis, cancer-associated thrombosis, autoimmune disorders, and others. This review summarizes the literature on what is known about platelets with different size and describes controversies of studies with large and small platelets putting a focus on their thrombogenicity, age, and on the association of MPV with the mentioned diseases.
Topics: Blood Platelets; Hemostasis; Humans; Mean Platelet Volume; Thrombocytopenia; Thrombosis
PubMed: 32108994
DOI: 10.1111/jth.14788 -
Blood Jun 2022The inherited thrombocytopenia syndromes are a group of disorders characterized primarily by quantitative defects in platelet number, though with a variety demonstrating... (Review)
Review
The inherited thrombocytopenia syndromes are a group of disorders characterized primarily by quantitative defects in platelet number, though with a variety demonstrating qualitative defects and/or extrahematopoietic findings. Through collaborative international efforts applying next-generation sequencing approaches, the list of genetic syndromes that cause thrombocytopenia has expanded significantly in recent years, now with over 40 genes implicated. In this review, we focus on what is known about the genetic etiology of inherited thrombocytopenia syndromes and how the field has worked to validate new genetic discoveries. We highlight the important role for the clinician in identifying a germline genetic diagnosis and strategies for identifying novel causes through research-based endeavors.
Topics: Blood Platelets; High-Throughput Nucleotide Sequencing; Humans; Platelet Count; Syndrome; Thrombocytopenia
PubMed: 35167650
DOI: 10.1182/blood.2020009300 -
Blood May 2023
Topics: Humans; Thrombocytopenia; Blood Platelet Disorders; Ribosomes
PubMed: 37140956
DOI: 10.1182/blood.2023019949 -
Clinical Toxicology (Philadelphia, Pa.) Jul 2014Abstract A summary of heparin-induced thrombocytopenia (HIT) is presented. HIT is an adverse drug reaction characterized by thrombocytopenia and a high risk for venous... (Review)
Review
Abstract A summary of heparin-induced thrombocytopenia (HIT) is presented. HIT is an adverse drug reaction characterized by thrombocytopenia and a high risk for venous or arterial thrombosis. The frequency of HIT ranges from 1 to 5% of patients receiving heparin with exact frequencies ranging between specific agents. Interestingly, this immune-mediated syndrome is ironically associated with thrombosis, not bleeding, with thrombin formation playing a major role. It is caused by heparin-dependent, platelet-activating antibodies that identifies a self-protein, PF4, bound to heparin that results in an antibody formation. The resulting platelet activation is associated with increased thrombin generation. Typically, the platelet count fall begins 5-10 days after starting heparin, although a rapid platelet count fall can occur in a patient who has antibodies from recent heparin use. Typical causes of HIT as well as the best diagnostic studies and treatment are discussed in this review. HIT was reviewed using a pubmed™ search; google scholar™ using key words: "Heparin-induced thrombocytopenia"; "heparin", and "drug AND thrombocytopenia."
Topics: Antibodies; Heparin; Humans; Thrombocytopenia
PubMed: 24844576
DOI: 10.3109/15563650.2014.917181 -
Haematologica Jun 2022The new techniques of genetic analysis have made it possible to identify many new forms of inherited thrombocytopenias (IT) and study large series of patients. In recent... (Review)
Review
The new techniques of genetic analysis have made it possible to identify many new forms of inherited thrombocytopenias (IT) and study large series of patients. In recent years, this has changed the view of IT, highlighting the fact that, in contrast to previous belief, most patients have a modest bleeding diathesis. On the other hand, it has become evident that some of the mutations responsible for platelet deficiency predispose the patient to serious, potentially lifethreatening diseases. Today's vision of IT is, therefore, very different from that of the past and the therapeutic approach must take these changes into account while also making use of the new therapies that have become available in the meantime. This review, the first devoted entirely to IT therapy, discusses how to prevent bleeding in those patients who are exposed to this risk, how to treat it if it occurs, and how to manage the serious illnesses to which patients with IT may be predisposed.
Topics: Blood Coagulation Disorders; Blood Platelets; Humans; Mutation; Thrombocytopenia
PubMed: 35642487
DOI: 10.3324/haematol.2022.280856 -
Hepatology Communications Feb 2022Liver injury, characterized predominantly by elevated aspartate aminotransferase and alanine aminotransferase, is a common feature of coronavirus disease 2019 (COVID-19)... (Review)
Review
Liver injury, characterized predominantly by elevated aspartate aminotransferase and alanine aminotransferase, is a common feature of coronavirus disease 2019 (COVID-19) symptoms caused by severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2). Additionally, SARS-CoV-2 infection is associated with acute-on-chronic liver failure in patients with cirrhosis and has a notably elevated mortality in patients with alcohol-related liver disease compared to other etiologies. Direct viral infection of the liver with SARS-CoV-2 remains controversial, and alternative pathophysiologic explanations for its hepatic effects are an area of active investigation. In this review, we discuss the effects of SARS-CoV-2 and the inflammatory environment it creates on endothelial cells and platelets more generally and then with a hepatic focus. In doing this, we present vascular inflammation and thrombosis as a potential mechanism of liver injury and liver-related complications in COVID-19.
Topics: Blood Platelet Disorders; COVID-19; Endothelium, Vascular; Humans; Inflammation; Liver Diseases; Thrombosis
PubMed: 34658172
DOI: 10.1002/hep4.1843 -
Advances in Clinical and Experimental... 2016Acquired von Willebrand syndrome is a rare hemorrhagic diathesis, with clinical symptoms similar to those associated with the inherited form von Willebrand disease. This... (Review)
Review
Acquired von Willebrand syndrome is a rare hemorrhagic diathesis, with clinical symptoms similar to those associated with the inherited form von Willebrand disease. This syndrome is characterized by a lack of previous bleeding symptoms, negative familial history, and occurrence in a relatively older age. Most commonly, acquired von Willebrand syndrome develops in the course of other conditions, such as lymphoproliferative, myeloproliferative, cardiovascular and autoimmune disorders; additionally, it can be associated with some non-hematological malignancies and use of certain prescription drugs. Pathogenesis of von Willebrand syndrome is complex and not fully understood. Deficiency or impaired activity of von Willebrand factor can result from the presence of specific antibodies against this factor, its adsorption onto the surfaces of neoplastic cells, mechanic injury or proteolysis. Diagnosis is based on the measurements of plasma concentration and the activity of von Willebrand factor and multimer analysis. Management of acquired von Willebrand syndrome includes the therapy of the underlying disease and the control or prevention of bleeding. Hemostatic drugs that are most commonly prescribed in this syndrome include desmopressin, von Willebrand factor concentrates, recombinant activated factor VII, intravenous immunoglobulin and adjunctive antifibrinolytic therapy. Additionally, plasmapheresis is required in some cases.
Topics: Hemorrhage; Humans; von Willebrand Diseases; von Willebrand Factor
PubMed: 28028990
DOI: 10.17219/acem/64942 -
Haematologica Feb 2021Inherited platelet disorders resulting from platelet function defects and a normal platelet count cause a moderate or severe bleeding diathesis. Since the description of... (Review)
Review
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 thrombasthenia resulting from defects of ITGA2B and ITGB3, new inherited platelet disorders have been discovered, facilitated by the use of high throughput sequencing and genomic analyses. Defects of RASGRP2 and FERMT3 responsible for severe bleeding syndromes and integrin activation have illustrated the critical role of signaling molecules. Important are mutations of P2RY12 encoding the major ADP receptor causal for an inherited platelet disorder with inheritance characteristics that depend on the variant identified. Interestingly, variants of GP6 encoding the major subunit of the collagen receptor GPVI/FcRγ associate only with mild bleeding. The numbers of genes involved in dense granule defects including Hermansky-Pudlak and Chediak Higashi syndromes continue to progress and are updated. The ANO6 gene encoding a Ca2+-activated ion channel required for phospholipid scrambling is responsible for the rare Scott syndrome and decreased procoagulant activity. A novel EPHB2 defect in a familial bleeding syndrome demonstrates a role for this tyrosine kinase receptor independent of the classical model of its interaction with ephrins. Such advances highlight the large diversity of variants affecting platelet function but not their production, despite the difficulties in establishing a clear phenotype when few families are affected. They have provided insights into essential pathways of platelet function and have been at the origin of new and improved therapies for ischemic disease. Nevertheless, many patients remain without a diagnosis and requiring new strategies that are now discussed.
Topics: Blood Platelet Disorders; Blood Platelets; Genotype; Guanine Nucleotide Exchange Factors; Humans; Phenotype; Thrombasthenia
PubMed: 33147934
DOI: 10.3324/haematol.2020.248153 -
Hematology. American Society of... Dec 2016This review will discuss how 2 common and morbid conditions, renal disease and liver disease, alter platelet number and function. It will review the impact of... (Review)
Review
This review will discuss how 2 common and morbid conditions, renal disease and liver disease, alter platelet number and function. It will review the impact of thrombocytopenia on bleeding complications in patients with these disorders and whether the low platelet count actually correlates with bleeding risk. Emerging data also suggest that platelets are much more than bystanders in both renal and liver disease, but instead play an active role in the pathobiology of these disorders. This review will briefly cover the emerging information on novel roles of platelets in the biology of renal and liver disease.
Topics: Blood Platelets; Hemorrhage; Humans; Kidney Diseases; Liver Diseases; Thrombocytopenia
PubMed: 27913488
DOI: 10.1182/asheducation-2016.1.251