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Recombinant Factor VIIa Prophylaxis in 2 Brothers with Bernard-Soulier Syndrome.

BACKGROUND Bernard-Soulier Syndrome (BSS) is a rare autosomal recessive platelet disorder characterized by thrombocytopenia, giant platelets, prolonged bleeding time, and defective platelet adhesion due to abnormalities in the glycoprotein 1b-IX-V complex. Management of BSS is challenging due to the risk of severe bleeding and complications associated with repeated platelet transfusions. Recombinant activated factor VIIa (rFVIIa, trade name: NovoSeven) has emerged as a potential alternative therapy. CASE REPORT We report the cases of 2 brothers with BSS who experienced recurrent mucocutaneous …

Retrospective comparison of maternal vs. HPA-matched donor platelets for treatment of fetal alloimmune thrombocytopenia.

BACKGROUND AND OBJECTIVES: In fetal alloimmune thrombocytopenia (FAIT), transplacental maternal antibodies cause destruction of fetal platelets. FAIT is similar to fetal Rhesus haemolytic disease, but half of the affected fetuses are born to primiparous women. In 10-20% of cases, prenatal and perinatal intracranial haemorrhages are reported. Different therapeutic approaches have been described, including maternally administered high-dose intravenous immunoglobulin (high dose IVIG) without or with steroids or intrauterine transfusion (IUT) of compatible platelets. For the latter, the use of pla

Neurosurgical bleeding in platelet storage pool disorder: a case report.

Dense-granule deficiency (DGD) is an inherited platelet disorder due to the absence of dense granules essential for activation of platelets … res often receive tranexamic acid (TXA), desmopressin, and/or human-leukocyte antigen (HLA)-matched platelet transfusions. We review the clinical management of intracranial tumor surgery, as well as Cyberknife radiosurgery, in our patient with DGD. After diagnosis was known, thoughtful hemostatic planning with empiric platelet transfusions and TXA prevented recurrent …

Platelets mediate trained immunity against bone and joint infections in a mouse model.

AIMS: Trained immunity confers non-specific protection against various types of infectious diseases, including bone and joint infection. Platelets are active participants in the immune response to pathogens and foreign substances, but their role in trained immunity remains elusive. METHODS: We first trained the innate immune system of C57BL/6 mice via intravenous injection of two toll-like receptor agonists (zymosan and lipopolysaccharide). Two, four, and eight weeks later, we isolated platelets from immunity-trained and control mice, and then assessed whether immunity training altered platel

Neural network reveals platelet age from fluorescence microscopy images.

Platelets are small, anucleate cells with a primary physiological role in vascular damage repair (hemostasis) and initiation of thrombus formation in response to vascular injury. Platelets circulate approximately 7-10 days, slowly undergoing age-related changes in molecular composition, morphology, activation capacity, function, and surface receptor density. As older platelets are associated with poor clinical outcome, no in vitro tests are available to predict platelet age, or to determine the fitness of platelet transfusion products. In this study, we developed a convolutional neural network…

Platelet-derived factor V promotes angiogenesis in a mouse hind limb ischemia model.

OBJECTIVE: Coagulation factor V (FV) is distributed in plasma and platelet pools, which are distinguished by physical and functional differences. FV has been extensively studied for its roles in coagulation. The roles of FV in other physiologic pathways remain understudied. METHODS: Hind limb ischemia was produced in transgenic mice by femoral artery ligation, with different levels of FV gene expression restricted to the plasma or platelets. RESULTS: Hind limb blood flow perfusion in mice with higher platelet FV was significantly increased. The expression of major angiogenesis-related factor

An HPA-1a-positive platelet-depleting agent for prevention of fetal and neonatal alloimmune thrombocytopenia: a randomized, single-blind, placebo-controlled, single-center, phase 1/2 proof-of-concept study.

BACKGROUND: Fetal/neonatal alloimmune thrombocytopenia (FNAIT) is a rare and potentially life-threatening bleeding disorder of the fetus/new … o 1 hour after transfusion of HPA-1ab platelets. Cohort 1B received RLYB211 or placebo, followed by platelet transfusion 1 week later. Primary endpoint was the half-life of transfused platelets in circulation after administration of RLYB211 or placebo, determined by flow cytometry. Proof of concept was ≥90% reduction of half-life relative to placebo. RESULTS: Twelve …

Managing Anti-Platelet Therapy in Thrombocytopaenic Patients with Haematological Malignancy: A Multinational Clinical Vignette-Based Experiment.

Data on anti-platelet therapy (APT) for prevention of atherothrombotic events in thrombocytopaenic cancer patients is lacking. We aimed to identify patient and physician characteristics associated with APT management in thrombocytopaenic patients with haematological malignancy. A clinical vignette-based experiment was designed. Eleven haematologists were interviewed, identifying five variable categories. Next, 18 hypothetical vignettes were generated. Each physician received three vignettes and chose to: hold all APT; continue APT without platelet transfusion support; or continue APT with plat…

Open-label pilot study of romiplostim for thrombocytopenia after autologous hematopoietic cell transplantation.

There are no standard treatments to prevent or hasten the recovery from severe conditioning-regimen-induced thrombocytopenia occurring after autologous hematopoietic cell transplantation (auto-HCT). We conducted an open-label, single-arm pilot study of romiplostim, a thrombopoietin receptor agonist, to enhance platelet recovery in patients with multiple myeloma or lymphoma undergoing auto-HCT. All patients were treated weekly with romiplostim starting day +1 after auto-HCT until the platelet count was >50 × 109/L without transfusion. Compared with contemporary retrospective data from romiplost

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… Biostatistical analyses The primary endpoint was the number of days requiring platelet transfusions or with grade 4 thrombocytopenia after auto-HCT. … Romiplostim-treated patients with MM had a median number of days of platelet transfusions or grade 4 thrombocytopenia of 6 (range, 0-17), and the median number of platelet transfusions received per patient during the auto-HCT was 2 (range, 0-7). …

Recombinant human thrombopoietin improves platelet engraftment after autologous hematopoietic stem cell transplantation in patients with aggressive lymphoma

High-dose chemotherapy (HDT) followed by autologous hematopoietic stem cell transplantation (ASCT), recommended as consolidation therapy for … latelet engraftment increases the risk of bleeding events, even life-threatening, and also leads to platelet transfusion dependence or resistance accompanied by the increasing incidence of transfusion side effects [5]. All of the above results in prolonged hospital stay, increased medical cost and decreased quality of life for patients [6-8]. However, there is no effe …

The German Haemovigilance System--reports of serious adverse transfusion reactions between 1997 and 2007.

Data of the German Haemovigilance System were collected from 1997 to 2007 and assessed on the basis of pre-defined safety standards. Suspected cases of serious adverse reactions following transfusions reported to the Paul-Ehrlich-Institut were evaluated on the basis of national criteria, and the definitions of International Society of Blood Transfusion (ISBT) in compliance with defined causality criteria. The suspected cases were rated as confirmed and unconfirmed transfusion reactions. Assessment of causality took into consideration the clinical course of the adverse reaction and, if necessar

Approaches to minimize infection risk in blood banking and transfusion practice.

The use of blood donor history and state-of-the-art FDA-licensed serological and nucleic acid testing (NAT) assays have greatly reduced the … ble to test for the presence of lipotechoic acid and lipopolysaccharide bacterial products prior to platelet transfusion. Attention has now turned to emerging infectious diseases including variant Creutzfeldt-Jakob disease, dengue, babesiosis, Chagas' disease and malaria. Challenges are presented to identify and prevent transmission of these agents. Several methods ar …

[Efficacy Prediction of Platelet Count Trajectories after Induction Therapy with Venetoclax Combined with Azacitidine in Newly Diagnosed AML Patients].

OBJECTIVE: To investigate platelet count trajectories after induction therapy with venetoclax combined with azacitidine (VA regimen) in newl … tic regression analysis showed that IDH1/2 mutation (OR =3.908, 95%CI : 1.023-14.923, P =0.046) and platelet transfusion (OR =0.771, 95%CI : 0.620-0.959, P =0.020) were independent influencing factors of platelet trajectory. CONCLUSION: The dynamic trajectory of platelet counts in newly diagnosed AML patients who received VA treatment can serve as a significant indic …

CREST: clinical bleeding and risk evaluation in hematology-oncology patients: a systematic review and meta-analysis of thromboelastography's role.

Thrombocytopenia and bleeding are common complications of hematologic malignancies. Often, prophylactic platelets are administered to minimize bleeding risk, based on total platelet count (TPC). However, TPC is a poor predictor, and does not provide rapid information. This review presents a novel prospective in the use of point-of-care viscoelastic studies to assess bleeding risk and guide transfusion therapy in a haematological oncological population, where its use can be extended to a ward level as a bedside test. Monitoring TEG maximum amplitude trends may be useful to guide transfusion pro

[Clinical analysis of 60 cases of pregnancy with thrombocytopenia].

Sixty cases of pregnancy with thrombocytopenia were analyzed. Bleeding syndrome was found in 36 patients during pregnancy, with 7 cases suff … uring delivery, as well as the incidence of thrombocytopenia in the newborn infant use of excessive platelet transfusion is not encouraged, since it implicates an increase in antibody-induction (PAIG) and probability of C type hepatitis in the recipient. Platelet transfusion should be reserved only for the treatment of serious hemorrhage. There were 53 newborn infants …

Comparative evaluation of two autotransfusion devices in a 72h survival swine model of surgically induced controlled splenic bleeding.

Autotransfusion is a key strategy in hemorrhagic surgical procedures, reducing risks like disease transmission and immunosuppression due to allogenic transfusion. While conventional devices efficiently process red blood cells by centrifugation, they don't address complications requiring additional platelet transfusions. The innovative same™ device (i-SEP, France), utilizing hollow-fiber filtration, preserves both red blood cells and platelets without damaging cell integrity. This study designed as a prospective randomized controlled trial compared cell concentration and washout performances of…

Pilot study of HLA alloimmunization after transfusion with pre-storage leucodepleted blood products in aplastic anaemia.

We have performed a pilot study to examine the incidence of alloimmunization using pre-storage leucocyte-depleted blood products (PLDP) in 16 previously transfused aplastic anaemia (AA) patients with no detectable HLA antibodies. A further eight AA patients with HLA antibodies received HLA-matched PLDP. Leucodepleted apheresed platelets were obtained using either Cobe spectra or Haemonetics system with an integral pall filter. Pall BPF4 filters were used for red cell preparation. Patients' sera were tested for HLA antibodies using lymphocytotoxicity (LCT). Patients who were HLA antibody negati

Neonatal platelets differentiate monocytes to a myeloid-derived suppressor cell phenotype.

Adult platelets are relatively enriched in immune-related molecules compared with neonatal platelets, but neonatal platelets express some gr … tal period by indirectly limiting T-cell responses, perhaps contributing to the adverse outcomes of platelet transfusions to neonates.

Acute pain transfusion reaction.

A 34-year-old woman with a diagnosis of hemophagocytic lymphohistocytosis (HLH) received a double umbilical cord blood transplantation following a myeloablative chemotherapy preparative regimen with busulfan and cyclophosphamide. HLH is a rare, potentially fatal hematologic disorder characterized by the overactivation of histocytes and T lymphocytes, leading to organ infiltration and acute illness. On day 25 post-transplantation, the patient required a platelet transfusion for a platelet count of 6,000 per ml (normal range = 150,000-450,000 per ml). The patient's blood type prior to the cord b…

Viscoelastic Testing Prior to Non-surgical Procedures Reduces Blood Product Use Without Increasing Bleeding Risk in Cirrhosis.

BACKGROUND/AIMS: Thromboelastography (TEG) and Rotational Thromboelastometry (ROTEM) analyze hemostatic function in patients with coagulopat … in adult patients with cirrhosis were included. Primary outcomes were fresh frozen plasma (FFP) and platelet transfusion and secondary outcomes of post-procedure bleeding, transfusion-related complications, and mortality; and were reported as standardized mean differences (SMD) and risk ratios (RR). RESULTS: Six studies (five randomized controlled trials and one coho …