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Desmopressin in inherited disorders of platelet function.

Following the first clinical use in haemophilia and von Willebrand disease in 1977, the synthetic analogue of vasopressin 1-deamino-8-D-arginine vasopressin (DDAVP, desmopressin) was successfully employed for the management of a series of bleeding disorders, including congenital and acquired defects of platelet function. In this setting, few haemostatic approaches are available and, in particular for severe bleeding and major invasive procedures, the transfusion of platelet concentrates is the first-choice treatment. Therefore, DDAVP was (and remains) an attractive therapeutic alternative, bei

Blood transfusion in orthotopic liver transplantation: six-year experience.

Patients undergoing orthotopic liver transplantation (OLT) are susceptible to massive blood loss and require transfusion. Possible reasons for increased transfusion demands include platelet abnormalities, thrombocytopenia secondary to hypersplenism, clotting factor deficiencies, fibrinolysis, increased surgical blood loss associated with portal hypertension and previous surgical procedures, and hypothermia. The purpose of this study was to review trends in blood product usage during our first 6 years of experience performing OLT.

The effect of plasma depletion of platelet concentrates on platelet aggregation and phosphatidylserine expression.

Plasma-reduced platelet concentrates are commonly administered to prevent febrile transfusion reactions and to avoid fluid overload in neonates. Because little is known about the influence of centrifugation and resuspension on functional aspects of platelets, we examined the effects of plasma-reduction on platelet aggregation and platelet-dependent thrombin generation. Our results show that plasma reduction and resuspension of the platelet pellet in saline or plasma results in a significant reduction in platelet aggregation to a combination of the platelet agonists adenosine diphosphate and ep

Platelet and Fibrinogen Contribution to Clot Strength in Premature Neonates with Sepsis.

Background/Objectives: Platelet transfusions are administered to preterm neonates with thrombocytopenia prophylactically to decrease their bleeding risk. The amplitude difference between the extrinsic rotational thromboelastometry (EXTEM) and the fibrinogen rotational thromboelastometry (FIBTEM) assays is considered an index of platelet contribution to clot strength, guiding transfusion management. The difference in maximum clot elasticity (MCE) (namely the platelet contribution to clot elasticity-MCEplatelet) is considered highly accurate. Limited data exist to specify the contribution of pla…

Mitochondrial pyruvate dehydrogenase kinases contribute to platelet function and thrombosis in mice by regulating aerobic glycolysis.

Resting platelets rely on oxidative phosphorylation (OXPHOS) and aerobic glycolysis (conversion of glucose to lactate in the presence of oxygen) for their energy requirements. In contrast, platelet activation exhibits an increased rate of aerobic glycolysis relative to OXPHOS. Mitochondrial enzymes pyruvate dehydrogenase kinases (PDKs) phosphorylate the pyruvate dehydrogenase (PDH) complex to inhibit its activity, thereby diverting the pyruvate flux from OXPHOS to aerobic glycolysis upon platelet activation. Of 4 PDK isoforms, PDK2 and PDK4 (PDK2/4) are predominantly associated with metabolic

A Novel Approach to Essential Thrombocythemia and Cardiac Surgery.

Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by persistent thrombocytosis (>450 × 109/L). Patients with ET are at risk for thrombotic or hemorrhagic complications especially during cardiovascular operations. We discuss a patient with ET requiring coronary artery bypass grafting with a presenting platelet count of 1631 × 109/L. Platelet reduction with an intraoperative autologous transfusion technique decreased the platelet count to 758 × 109/L before initiation of cardiopulmonary bypass. In addition, perioperative cangrelor therapy was administered to further l

Рекомбинантный тромбопоэт не способствует пересадке тромбоцитов у недавно диагностированных пациентов с несколькими миеломой после аутологической трансплантации стволовых клеток.

Оценка воздействия рекомбинантного тромбопоэтина человека (rhTPO) на пересадку тромбоцитов после аутологической трансплантации стволовых клеток (ASCT) у недавно диагностированных пациентов с множественными миеломами (NDMM). Клинические данные 40 Пациенты NDMM, подвергшиеся повышенному кондиционированию с использованием мельфалана и ACT, были проанализированы с помощью 26 получение rhTPO и 14 в контрольной группе. Основным конечным моментом исследования было среднее время для пересадки тромбоцитов. К числу конечных точек вторичного исследования относятся среднее время нейтрофильной эграции, кол

Совпадение в аннотации оригинала
To evaluate the effect of recombinant human thrombopoietin (rhTPO) on platelet engraftment after autologous stem cell transplantation (ASCT) … e groups. Administering rhTPO after ASCT for NDMM did not accelerate platelet engraftment or reduce platelet transfusions, but increased hospitalization costs.

Aggressive combination therapy in the successful management of life-threatening intracranial hemorrhage in a patient with idiopathic thrombocytopenic purpura.

We describe acute therapy for a 13-year-old female from Panama with chronic idiopathic thrombocytopenia purpura (ITP) refractory to steroids, splenectomy, vinca alkaloids, and azathioprine. She presented with neurologic deterioration from a posterior fossa intracranial hemorrhage (ICH). This followed a 3-month history of severe dysfunctional uterine bleeding, progressive from menarche, which had required multiple red cell transfusions. Steroid and vinblastine therapy and transfusion of 40 U of platelets failed to increase the platelet count above 10,000/microliter. Development of a second larg

Transfusion medicine and the pregnant patient.

Alloimmunity in pregnancy is the basis for two of the major complications of pregnancy in transfusion medicine: hemolytic disease of the fetus and newborn (HDFN), and fetal and neonatal alloimmune thrombocytopenia (FNAIT). Use of Rh(D) immune globulin has dramatically reduced the incidence of HDFN in Rh(D)-mismatched pregnancies. Treatment of HDFN may involve intrauterine transfusion, with fetal and neonatal survival rates of 70% to 90%. Treatments for FNAIT include immune globulin, steroids, or in severe cases, intrauterine platelet transfusions. Transfusion medicine is central to the managem…

Post-transfusion purpura: pathogenic mechanisms and relationship with immune thrombocytopenia: a critical analytical review.

Post-transfusion purpura (PTP) is a rare, life-threatening immune-mediated adverse transfusion event. It is characterized by severe thrombocytopenia developing 5-10 days after transfusion of platelet-containing blood products. The most notable pathological paradox is that alloantibodies directed against foreign human platelet antigen (HPA) can destroy the patient's autologous platelets that do not express the corresponding target antigen. PTP predominantly affects middle-aged women, and its major clinical manifestation is mucocutaneous bleeding, with intracranial hemorrhage being the leading c

Prevalence and risk factors of antibodies to HLA according to different cut-off values of mean fluorescence intensity in haploidentical allograft candidates: A prospective study of 3805 subjects.

The importance of anti-HLA antibodies in transplantation settings, such as HLA-mismatched or haploidentical hematopoietic stem cell transpla … all cases. When the MFI cut-off value was ≥500 for positivity, multivariate analysis indicated that platelet transfusion, underlying disease, and pregnancy were the most important risk factors for the presence of anti-HLA antibodies for the total patients. Subgroup analysis according to age, gender, and underlying disease showed that pregnancy was the most important r …

Calcium carbonate polymorphs enhance coagulation in citrated blood via platelet concentration and calcium release.

Citrate, widely used as an anticoagulant in transfusion and extracorporeal therapies, disrupts calcium-dependent coagulation by chelating ionized calcium, resulting in hypocalcemia and severe coagulopathy. This interference elevates bleeding risks and may trigger hemorrhagic shock. Current treatments for local bleedings in the context of citrate-induced coagulopathy include topical hemostats that either provide a physical barrier to the bleeding or activate platelets and the coagulation cascade. This study investigates the potential of calcium carbonate (CaCO3) to counteract the citrate coagul

IL-8 and sCD40L predict platelet refractoriness and survival of pediatric patients with solid tumors receiving chemotherapy.

OBJECTIVE: To assess inflammation, endothelial damage, and platelet activation during platelet transfusion refractoriness (PTR) in pediatric patients with solid tumors. METHODS: A cohort of 36 PTR and 38 Non-PTR pediatric patients with solid tumor was enrolled. Patients received standard chemotherapy and platelet transfusion according to guidelines. Levels of inflammatory mediators (IL-1β and IL-8, etc.), endothelial damage marker (vWF), and platelet activation markers (sP-selectin, sCD40L and RANTES) in pre-transfusion plasma were quantified by flow cytometry and ELISA. Associations with PTR,…

Blood Product (Donor) Noninfectious and Infectious Testing and Modification.

Blood transfusion begins with safe donor selection and testing. In the United States, the blood supply and transfusion are highly regulated. Blood transfusion safety is multifaceted, whereby each of the elements of the blood safety value chain, spanning donor recruitment and qualification, to collection, blood processing, testing, transfusion practice, and posttransfusion surveillance, must be optimized to minimize risk. Pathogen inactivation is a promising approach to decrease bacterial contamination of platelets, inactivate parasites and viruses, and decrease risks associated with emerging a

Natural history of antiplatelet antibody formation in patients with Glanzmann thrombasthenia: a French multicenter prospective study.

BACKGROUND: Glanzmann thrombasthenia (GT) is a rare inherited platelet function disorder caused by defects in the ITGA2B or ITGB3 genes encoding the αIIbβ3 integrin. Repeated transfusions often lead to immunization against αIIbβ3 and/or HLA antigens, compromising transfusion efficacy. OBJECTIVES: This study aimed to prospectively characterize the development and evolution of anti-αIIbβ3 and anti-HLA antibodies in a large cohort of French patients with GT and to assess their potential functional effects on platelet activity. METHODS: This multicenter prospective study included 55 patients wit

The effects of pegylated recombinant human megakaryocyte growth and development factor (PEG-rHuMGDF) on platelet recovery in breast cancer patients undergoing autologous bone marrow transplantation.

OBJECTIVE: To assess the safety and efficacy of pegylated recombinant human megakaryocyte growth and development factor (PEG-rHuMGDF) admini … In the phase 1/2 study, the time to platelet recovery to greater than or equal to 20 x 10(9)/L and platelet transfusion requirements were significantly reduced for patients treated with PEG-rHuMGDF compared with placebo (p < 0.05). In the phase 3 study, no significant differences in the kinetics of early thrombopoiesis or platelet transfusions after ABMT were observe …

[Autoimmune thrombocytopenia during pregnancy (case report and review of literature)].

A case of autoimmune thrombocytopenia [AIT], diagnosed in the first trimester of pregnancy is described. Despite the active treatment with s … ation of rFVIIa (because of the tendency for elevation of the titers of allogenic antibodies due to platelet transfusions) and of antifibrinolytics (preserving platelet functions).

Acute bleeding complications in patients after bone marrow transplantation.

Acute bleeding is a frequent complication that commonly associates with increased morbidity after bone marrow transplantation. Except for di … ay identify patients at risk for bone marrow transplantation toxicity. Until recently, prophylactic platelet transfusions were commonly given at a trigger of 20 x 10(9)/L. Whereas bleeding is more likely to occur when platelet counts drop to low levels, most bleeding episodes were recorded with platelet counts greater than 20 x 10(9)/L, suggesting causes other than pr …

Platelet Count and Function during Pediatric Extracorporeal Membrane Oxygenation.

Extracorporeal membrane oxygenation (ECMO) is a form of life support used to treat neonates, children, and adults with cardiorespiratory failure refractory to conventional therapy. This therapy requires the use of anticoagulation to prevent clotting in the extracorporeal circuit, but anticoagulation also increases the risk of bleeding on ECMO. Both bleeding and thrombosis remain significant complications on ECMO and balancing these risks is challenging. Acquired platelet dysfunction is common during ECMO and quantitative and qualitative platelet dysfunction contributes to bleeding risk. Optima

Platelet in vitro assays: their correspondence with their in vivo hemostatic potential.

Developments during the past few years have resulted in multiple kinds of platelet products for transfusion. This involves different collection methods, containers, preservative solutions, modifications of storage temperatures and durations, and additional treatments such as pathogen reduction. Much experience has been obtained testing these processes in vitro to seek indications of in vivo effectiveness. Availability of an in vitro method that correlated with in vivo effectiveness would be extremely valuable for these different kinds of platelet products and as more innovation in platelet pre