We report the long-term evaluation over 12 years of a simplified technique for stemcell cryopreservation at -80ºC without rate-controlled freezing and with 5% (n=251) or 10% (n=47) DMSO as the sole cryoprotectant. Platelet recovery was greater in the 5% DMSO group while long-term hematologic recovery did not differ. Factors influencing a faster hematologic recovery were infusion of more than 2.7×10 6 /kg of CD34 + cells, 10% DMSO cryopreservation and G-CSF. We confirm that the procedure is feasible with a reduction in infusion-related toxicity from 60% using 5% DMSO. Differences in hematologic reconstitution were not clinically significant if a minimum of 1. 1 The toxic effects related to DMSO infusion are generally dose-related and while they are usually mild, they can become severe.2-4 HES is a relatively non-toxic drug but it is related with long-lasting pruritus 5 and osmotic nephrotoxicity. Cryopreservation protocols usually involve ratecontrolled freezing followed by the storage of the HSC in either the liquid or vapor phase of liquid nitrogen. These procedures are time consuming and require expensive computerassisted devices.
Older patients with haemophilia (PWH) face many challenges related not only to haemophilia but also to general comorbidities associated with ageing. This article discusses the clinical experience published about the high prevalence of diseases in older PWH. These conditions are managed in the general population by healthcare workers with little training in haemophilia. Haemophilic arthropathy is common in elderly PWH. Prophylaxis starting at an early age in sufficient dose regimens to prevent arthropathy did not occur in patients who are now older than around 40 yr. Many PWH above this age thus have limitations in their activities of daily life. Cardiovascular diseases have become increasingly common in the growing, ageing cohort of PWH. Lifestyle issues such as sexual dysfunction may be exacerbated by the medical issues and psychological problems associated with haemophilia. Hepatitis C virus is a leading problem in PWH. Coinfection with HIV accelerates the progression to end-stage liver disease. Acute and chronic renal failure is more common in adult PWH than in general population. Other comorbidities are reviewed. The evidence is scarce, so it is imperative to report any experience regarding the diagnosis and treatment of these entities, to improve the quality of life of older PWH.
The pathogen safety of blood/plasma-derived products has historically been a subject of significant concern to the medical community. Measures such as donor selection and blood screening have contributed to increase the safety of these products, but pathogen transmission does still occur. Reasons for this include lack of sensitivity/specificity of current screening methods, lack of reliable screening tests for some pathogens (e.g. prions) and the fact that many potentially harmful infectious agents are not routinely screened for. Methods for the purification/inactivation of blood/plasma-derived products have been developed in order to further reduce the residual risk, but low concentrations of pathogens do not necessarily imply a low level of risk for the patient and so the overall challenge of minimising risk remains. This review aims to discuss the variable level of pathogenic risk and describes the current screening methods used to prevent/detect the presence of pathogens in blood/plasma-derived products
Worldwide vaccination against SARS-CoV-2 has allowed the detection of hematologic autoimmune complications. Adverse events (AEs) of this nature had been previously observed in association with other vaccines. The underlying mechanisms are not totally understood, although mimicry between viral and self-antigens plays a relevant role. It is important to remark that, although the incidence of these AEs is extremely low, their evolution may lead to life-threatening scenarios if treatment is not readily initiated. Hematologic autoimmune AEs have been associated with both mRNA and adenoviral vector-based SARS-CoV-2 vaccines. The main reported entities are secondary immune thrombocytopenia, immune thrombotic thrombocytopenic purpura, autoimmune hemolytic anemia, Evans syndrome, and a newly described disorder, so-called vaccine-induced immune thrombotic thrombocytopenia (VITT). The hallmark of VITT is the presence of anti-platelet factor 4 autoantibodies able to trigger platelet activation. Patients with VITT present with thrombocytopenia and may develop thrombosis in unusual locations such as cerebral beds. The management of hematologic autoimmune AEs does not differ significantly from that of these disorders in a non-vaccine context, thus addressing autoantibody production and bleeding/thromboembolic risk. This means that clinicians must be aware of their distinctive signs in order to diagnose them and initiate treatment as soon as possible.
Introduction The Haemophilia Early Arthropathy Detection with Ultrasound (HEAD‐US) system and scoring scale has proven to be an accurate and time‐efficient imaging method for identifying joint damage in patients with haemophilia. Aim Observational, multicentre, cross‐sectional study conducted in 8 centres in Spain that assessed the joint status of adult patients with severe haemophilia A (SHA) using HEAD‐US. Methods Joint status of the elbow, knee and ankle was evaluated in adults with SHA receiving on‐demand (OD) treatment, or primary (PP), secondary (SP), tertiary (TP) or intermittent (IP) prophylaxis. Results Of the 95 patients enrolled, 87 received prophylaxis (6.3% PP, 38.9% SP, 43.2% TP and 3.2% IP). Mean age was 35.2 years, and 59% of patients had not undergone image testing in the last year. The HEAD‐US score was 0 in all joints in 6.3% of patients. The ankle was the most affected joint, regardless of treatment regimen. Patients receiving OD treatment, TP or IP had the overall worst scores, mainly in the ankles and elbows; a similar but milder profile was observed in patients on SP; and patients on PP had the best score in all joints. Conclusion Joint function may be effectively preserved in patients with SHA on PP, but OD treatment or later initiation of prophylaxis does not seem to prevent progression of arthropathy. Disease worsening was observed in patients OD, TP or IP, most often affecting ankles and elbows. Closer ultrasound imaging monitoring may improve management of these patients.
Patients with blood disorders (including leukaemia, platelet function disorders and coagulation factor deficiencies) or acute bleeding receive blood-derived products, such as red blood cells, platelet concentrates and plasma-derived products. Although the risk of pathogen contamination of blood products has fallen considerably over the past three decades, contamination is still a topic of concern. In order to counsel patients and obtain informed consent before transfusion, physicians are required to keep up to date with current knowledge on residual risk of pathogen transmission and methods of pathogen removal/inactivation. Here, we describe pathogens relevant to transfusion of blood products and discuss contemporary pathogen removal/inactivation procedures, as well as the potential risks associated with these products: the risk of contamination by infectious agents varies according to blood product/region, and there is a fine line between adequate inactivation and functional impairment of the product. The cost implications of implementing pathogen inactivation technology are also considered.
Abdi and colleagues reported important insights regarding the treatment history of non-severe hemophilia A (HA) patients, 1 including incidence of joint bleeds in a large, international, and multi-center non-severe HA cohort (the INSIGHT cohort). Repeated intraarticular bleeding episodes in hemophilia patients may lead to degenerative arthropathy, which is the most frequent complication in HA regardless of the degree of severity. Despite the interesting findings described on annualized bleeding rate in mild-to-moderate HA patients, the study by Abdi and colleagues did not provide information on the joint status. The early identification of joint damage is indeed crucial to prevent the progression to hemophilic arthropathy, and thus periodic monitorization of the joint status is recommended for non-severe HA patients. 2 However, subclinical intra-articular bleeds may be hardly noticeable by clinical examination, especially in milder forms of HA; hemarthrosis and abnormalities may not be present for many years after birth, joint health awareness is usually lower, and most clinical evidence is generally extrapolated from severe patients' data. 3Diagnostic imaging is necessary to identify subtle alterations, and MRI is the gold standard for joint evaluation. Nevertheless, access to this technique is limited and its low feasibility for regular or on-demand (OD) check-ups has led to the implementation of musculoskeletal ultrasound as a point-of-care technique, with several reports supporting its usefulness. 4 This has been associated with the development of Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US), 5 a scoring system which enables a low-cost and quick evaluation of the six major joints, namely left and right sided elbows (LE, RE), knees (LK, RK), and ankles (LA, RA). Here we report unpublished findings of a 28-adult male patient subset with moderate (n = 14) and mild (n = 14) HA, with median age of 42.5 [IQR 32-60.5] years (40.5 [IQR 30.0-49.0] and 51.0 [IQR 34.0-63.0] years for moderate and mild, respectively) from a previously performed cross-sectional study to assess joint status in Spain. 6Not surprisingly we found 19 patients non-subjected to any evaluation by imaging modalities over the last 12 months (85.7%
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