Transfusion-associated adverse events incidence and severity after the implementation of an active hemovigilance program with 24 h follow-up. A prospective cohort study.

Background: Hemovigilance (HV) is usually based on voluntary reports (passive HV). Our aim is to ascertain credible incidence, severity, and mortality of transfusion-associated adverse events (TAAEs) using an active HV program.

Study Design And Methods: Prospective cohort study to estimate transfusion risk after 46,488 transfusions in 5830 patients, using an active HV program with follow-up within the first 24 h after transfusion.

The Proven Versatility of CpTiCl.

In the last two decades, titanocene monochloride has been postulated as a monoelectronic transfer reagent capable of catalyzing an important variety of chemical transformations. In this Perspective, our contributions to this growing field of research are summarized and analyzed. Especially known have been our contributions in C-C bond formation reactions, hydrogen-atom transfer from water to radicals, and isomerization reactions, as well as the development of a catalytic cycle that has subsequently allowed the preparation of a great variety of natural terpenes.

Extravascular hemolysis and complement consumption in Paroxysmal Nocturnal Hemoglobinuria patients undergoing eculizumab treatment.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia characterized by complement-mediated intravascular hemolysis that is effectively treated with eculizumab. However, treatment responses are reported heterogeneous with some patients presenting residual hemolysis and requiring RBC transfusions. Recent reports have shown that both extravascular hemolysis and incomplete C5 blockade can explain these suboptimal hematological responses.

[New mutation in a young woman diagnosed with Niemann-Pick disease type C].

Background And Objetive: To describe a new molecular variant of Niemann-Pick disease type C (NPC) in a 27 year-old patient with splenomegaly and abolition of osteotendinous reflexes.

Material And Methods: NPC1 is the main gene with described mutation in NPC disease. Here we report a case with a new mutation, p.

[Spanish consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria].

Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal disorder of the haematopoietic progenitor cells due to a somatic mutation in theX-linked phosphatidylinositol glycan class A gene. The disease is characterized by intravascular haemolytic anaemia, propensity to thromboembolic events and bone marrow failure. Other direct complications of haemolysis include dysphagia, erectile dysfunction, abdominal pain, asthenia and chronic renal failure (65% of patients).

Paroxysmal nocturnal hemoglobinuria: a single Spanish center's experience over the last 40 yr.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disease. To date, many reviews and series have been described. We report the experience of our center by presenting a review of 56 PNH patient cases with an average age at diagnosis of 38 yr and follow-ups beginning at approximately 40 yr; the median survival rate was 11 yr.

[Paroxysmal nocturnal hemoglobinuria therapy with eculizumab: Spanish experience].

Background And Objectives: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal disease characterized by complement-mediated hemolysis, bone marrow failure and thrombosis. Eculizumab is a humanized monoclonal antibody that blocks the cytolytic component of the complement system by binding to complement C5.

Material And Methods: We report the results of eculizumab treatment in 25 PNH patients from different centers in Spain.

Results of a pilot study on the use of third-party donor mesenchymal stromal cells in cord blood transplantation in adults.

Background Aims: Cord blood (CB) transplants with co-infusion of third-party donor (TPD) mobilized hematopoietic stem cells (MHSC) have been shown to result in 'bridge' engraftment with prompt neutrophil recovery and high final rates of CB engraftment and full chimerism. This strategy overcomes the limitation posed by low cellularity of CB units for unrelated transplants in adults. Enhancement of adaptive immunity reconstitution without increasing risks of graft-versus-host disease (GvHD) is required to optimize results further.