353 research outputs found
Platelet Counting and Measurement of Platelet Dimensions
No full textSince the introduction of automated blood cell analyzers, platelet counting and platelet dimension measurements are greatly improved in accuracy and disposability. No doubts in considering their relevance evident in routine clinical practice, particularly in diagnosis and management of many bleeding and thrombotic imbalances. Besides the impedance-based one, the first performed, and to date the most diffused, several others automated counting methods have been developed making platelet recognition increasingly more accurate. Platelet counting is now obtained not only by size but also by density and surface glycoproteins’ expression. Although these technological improvements, spurious abnormalities of platelet count such as pseudothrombocytopenia and pseudothrombocytosis can still occur, posing serious diagnostic difficulties. Therefore, in few selected clinical settings, the manual microscopy techniques are still considered of great utility to correctly identify and enumerate platelets. Moreover, the widespread automated cell blood counters make today available additional platelet parameters like the mean platelet volume and the amount of young platelets, calculated as reticulated platelets or immature platelet fraction. Although methodological issues remain to be solved, the availability of these further platelets’ parameters can also drive effectively the diagnosis of common or rare platelet disorders such as immune thrombocytopenia and inherited thrombocytopenias. The relevance in diagnosis and prognosis of these parameters is still under investigations in a large number of platelet diseases and in disorders not primarily affecting platelets
Investigational drugs in thrombotic thrombocytopenic purpura.
No full textIntroduction: Familial and acquired, idiopathic, thrombotic thrombocytopenic purpura (TTP) are life-threatening thrombotic microangiopathies characterized by thrombocytopenia and microangiopathic hemolytic anemia deriving from a deficiency of a disintegrin-like and metalloprotease with thrombospondin repeats (ADAMTS)13 activity induced by genetic defects or autoantibodies. Although the introduction of plasma exchange in 1991 made these conditions curable, many patients still die from TTP and improved therapeutic approaches are, therefore, required. Areas covered: Based on recent progress in terms of etiology and pathogenesis of TTP, several innovative therapies have been proposed in the last few years. This review provides an overview of results obtained with the ?new? therapeutic options in the light of the ?old? treatments that have been already validated by clinical trials. Expert opinion: Plasma exchange and steroids are still the most effective treatments for TTP, but several new therapeutic approaches promise to further improve the prognosis of affected patients. Immunosuppressants stronger than steroids are expected to inhibit the production of anti-ADAMTS13 autoantibodies in acquired forms when the standard approach fails to reach this goal. Moreover, new antithrombotic drugs are expected to reduce the risk of thrombosis during the period required for inhibiting the production of autoantibodies. Finally, the possibility of replacing the infusion of fresh frozen plasma with recombinant ADAMTS13 could further improve the prognosis of TTP, especially in subjects with familial forms
Mean platelet volume for distinguishing between inherited thrombocytopenias and immune thrombocytopenia - response to Beyan
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Hereditary thrombocytopenias: A growing list of disorders
No full textThe introduction of high throughput sequencing (HTS) techniques greatly improved the knowledge of inherited thrombocytopenias (ITs) over the last few years. A total of 33 different forms caused by molecular defects affecting at least 32 genes have been identified; along with the discovery of new disease-causing genes, pathogenetic mechanisms of thrombocytopenia have been better elucidated. Although the clinical picture of ITs is heterogeneous, bleeding has been long considered the major clinical problem for patients with IT. Conversely, the current scenario indicates that patients with some of the most common ITs are at risk of developing additional disorders more dangerous than thrombocytopenia itself during life. In particular, MYH9 mutations result in congenital macrothrombocytopenia and predispose to kidney failure, hearing loss, and cataracts, MPL and MECOM mutations cause congenital thrombocytopenia evolving into bone marrow failure, whereas thrombocytopenias caused by RUNX1, ANKRD26, and ETV6 mutations are characterized by predisposition to hematological malignancies. Making a definite diagnosis of these forms is crucial to provide patients with the most appropriate treatment, follow-up, and counseling. In this review, the ITs known to date are discussed, with specific attention focused on clinical presentations and diagnostic criteria for ITs predisposing to additional illnesses. The currently available therapeutic options for the different forms of IT are illustrated
Innovation in the field of thrombocytopenias: achievements since the beginning of the century and promises for the future
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Prevalencia, evolución y sobrevida de pacientes infectados con VIH/SIDA en la Unidad de Terapia Intensiva Pediátrica en el Hospital Infantil de México Federico Gómez, durante un periodo de 10 años
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