Publications by authors named "Prata Ph"

Purpose: Graft-versus-host disease (GVHD) is a common complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). GVHD may affect several organs, including ocular manifestations, ranging from dry eye syndrome to sight-threatening corneal ulceration or perforation. Limited information is available about characteristics and treatments of ocular GVHD and its relation to general prognosis.

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  • First-line treatments for autoimmune systemic diseases (ARD) typically involve immunosuppressive drugs, but long-term use can lead to significant health risks.
  • Innovative therapies like mesenchymal stromal cells (MSCs) and Chimeric Antigen Receptor (CAR) T cell therapies are emerging as promising alternatives for severe or refractory cases of ARD.
  • A workshop by the French Speaking Society of Bone Marrow and Cell Transplantation focuses on establishing healthcare pathways and safety protocols for the deployment of MSCs and CAR-T therapies in ARD treatment, emphasizing patient safety and collaboration among specialists.
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  • In France, the transfusion policy for patients undergoing allogeneic hematopoietic stem cell transplantation (alloHSCT) involves giving red blood cells (RBC) matching the donor's Rh type, which poses a risk of allo-immunization to both donors and recipients.* -
  • A study spanning 8 years revealed a 7.1% incidence of Rh incompatibility in 1012 alloHSCT cases, with 10.3% of evaluable patients developing alloantibodies, but none targeted the mismatched Rh antigens.* -
  • Although no immune-mediated hemolytic anemia occurred as a result of allo-immunization, a significant association was found between anti-RBC allo-immunization and
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Systemic sclerosis (SSc) is a rare autoimmune disease (AD), characterised by early diffuse vasculopathy, activation of the immune response and progressive skin and internal organ fibrosis. In severe progressive diffuse SSc (dSSc), autologous hematopoietic stem cell transplantation (aHSCT) improves survival, despite its own risk of complications and transplant related mortality (TRM). We present herein the case of a dSSc patient undergoing aHSCT with low dose cyclophosphamide conditioning and sudden acute myopericarditis and cardiogenic shock, four weeks after a second mRNA SARS-CoV-2 vaccine (Pfizer) injection.

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  • Patients with Fanconi anemia (FA) show chromosome instability, leading to exhaustion of hematopoietic stem cells and a higher risk of developing poor-prognosis myeloid leukemia.
  • A study involving 62 patients revealed unique mutations and structural variants that resemble BRCA-related cancers, with many patients showing chromosome 1q gain linked to MDM4 trisomy, which downregulates p53 signaling.
  • MDM4 triplication not only enhances the survival of FA stem cells but also promotes leukemia development, suggesting that targeting MDM4 could be a potential therapeutic strategy to disrupt this pathway.
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Patients with paroxysmal nocturnal hemoglobinuria (PNH) are susceptible to complement-mediated intravascular hemolysis and thrombosis. Factor H (FH) is the main regulator of the complement alternative pathway, which protects cells from unwanted complement-mediated damage. Although FH is not a glycosylphosphatidylinositol-linked molecule, it may play a role in PNH.

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Purpose: Secondary myeloid neoplasms (sMNs) remain the most serious long-term complications in patients with aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH). However, sMNs lack specific predictors, dedicated surveillance measures, and early therapeutic interventions.

Patients And Methods: We studied a multicenter, retrospective cohort of 1,008 patients (median follow-up 8.

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The management of cancer-associated thrombosis (CAT) poses unique challenges to healthcare professionals. While low-molecular weight heparins (LMWHs) have long been the gold standard for both the primary and secondary prevention of CAT, results from large randomized controlled trials assessing the benefit of direct oral anticoagulants (DOACs) in both settings have resulted in some paradigm shifts. Herein, we review and compare recommendations from the latest authoritative clinical practice guidelines (CPGs) for the management of CAT and summarize the most recent evidence on available treatment options.

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  • * The 2022 guidelines include specific treatments for patients with cancer and COVID-19, emphasizing low-molecular-weight heparins (LMWHs) and direct oral anticoagulants for managing blood clots.
  • * Key recommendations highlight the use of LMWHs or direct oral anticoagulants for at least 6 months, extended post-surgery prophylaxis, and primary prevention in certain cancer patients undergoing treatment.
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  • International guidelines now support the use of direct oral anticoagulants (DOACs) as a viable alternative to low-molecular-weight heparins (LMWHs) for treating cancer-associated thrombosis (CAT).
  • An updated meta-analysis of six randomized controlled trials involving 3,690 patients showed that DOACs significantly reduce the risk of CAT recurrence compared to LMWHs.
  • While DOACs have a slight increase in the risk of major and clinically relevant nonmajor bleeding, they do not affect overall mortality rates in cancer patients.
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Steroid-refractory graft versus host disease (GVHD) represents a fearsome complication after allogeneic hematopoietic stem cell transplantation (HSCT). We conducted a retrospective study on outcomes and risk factors associated with acute and chronic steroid-refractory GVHD in a large cohort of 1207 patients receiving HSCT in Saint Louis Hospital between 2007 and 2017. Among patients who developed an acute and/or a chronic GVHD, the cumulative incidences of acute and chronic steroid-refractory disease were 31% and 48%, respectively, at day +100 and 1-year post-HSCT.

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In the absence of an HLA-matched donor, the best treatment for acquired aplastic anemia patients refractory to immunosuppression is unclear. We collected and analyzed data from all acquired aplastic anemia patients who underwent a haploidentical transplantation with posttransplant cyclophosphamide in Europe from 2011 to 2017 (n = 33). The cumulative incidence of neutrophil engraftment was 67% (CI: 51-83%) at D +28 and was unaffected by age group, stem cell source, ATG use, or Baltimore conditioning regimen.

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Post-transplantation lymphoproliferative disease (PTLD) is a serious complication associated with Epstein-Barr virus (EBV) infection after hematopoietic stem cell transplantation (HSCT). Although anti-CD-20 therapy is now used as a preemptive strategy for EBV reactivation, PTLD still occurs in some patients. Here we analyzed outcomes and risk factors associated with PTLD transformation in 208 HSCT recipients who were diagnosed with EBV-DNAemia and received at least 1 course of rituximab.

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Prognosis of patients with graft failure is dismal, and retransplantation is the sole option for long-term survival. To address the interest of haploidentical transplantation as a salvage option in this context, we analyzed data from 24 patients with graft failure or loss retransplanted with a haploidentical donor who received post-transplant cyclophosphamide (PTCy) as graft-versus-host disease prophylaxis (GVHD). Fludarabine-based reduced-intensity conditioning was used in 23 patients and the Baltimore regimen in 14 patients.

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The bone marrow failure (BMF) syndromes are a group of rare disorders characterized by ineffective hematopoiesis resulting from deficiencies in the hematopoietic stem cell compartment. Although these diseases are typically acquired, some forms (e.g.

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Most studies of immunosuppressive therapy (IST) in myelodysplastic syndromes (MDS) are limited by small numbers and their single-center nature, and report conflicting data regarding predictors for response to IST. We examined outcomes associated with IST and predictors of benefit in a large international cohort of patients with MDS. Data were collected from 15 centers in the United States and Europe.

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Background: Acute schistosomiasis is a systemic hypersensitivity reaction against the migrating schistosomula and eggs. In this report, we describe an atypical outbreak of the disease with severe cases. Transmission occurred in a nonendemic area of Brazil, which became a new focus of transmission due to the in-migration of infected workers.

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Purpose: To evaluate the histopathology alterations of the intestinal mucosa of rabbits submitted to different times of mesenteric artery ischemia and reperfusion with and without celiac artery collateral circulation supply.

Methods: Two groups of eight male New Zealand white rabbits (weight 2.2-3.

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