Transfusion Medicine and Hemotherapy最新文献

[This corrects the article DOI: 10.1159/000533624.].

Background: Telomeres are the end-capping structures of all eukaryotic chromosomes thereby protecting the genome from damage and degradation. During the aging process, telomeres shorten continuously with each cell division until critically short telomeres prevent further proliferation whereby cells undergo terminal differentiation, senescence, or apoptosis. Premature aging due to critically short telomere length (TL) can also result from pathogenic germline variants in the telomerase complex or related genes that typically counteract replicative telomere shortening in germline and certain somatic cell populations, e.g., hematopoetic stem cells. Inherited diseases that result in altered telomere maintenance are summarized under the term telomere biology disorder (TBD).

Summary: Since TL both reflects but more importantly restricts the replicative capacity of various human tissues, a sufficient telomere reserve is particularly important in cells with high proliferative activity (e.g., hematopoiesis, immune cells, intestinal cells, liver, lung, and skin). Consequently, altered telomere maintenance as observed in TBDs typically results in premature replicative cellular exhaustion in the respective organ systems eventually leading to life-threatening complications such as bone marrow failure (BMF), pulmonary fibrosis, and liver cirrhosis.

Key messages: The recognition of a potential congenital origin in approximately 10% of adult patients with clinical BMF is of utmost importance for the proper diagnosis, appropriate patient and family counseling, to prevent the use of inefficient treatment and to avoid therapy-related toxicities including appropriate donor selection when patients have to undergo stem cell transplantation from related donors. This review summarizes the current state of knowledge about TBDs with particular focus on the clinical manifestation patterns in children (termed early onset TBD) compared to adults (late-onset TBD) including typical treatment- and disease course-related complications as well as their prognosis and adequate therapy. Thereby, it aims to raise awareness for a disease group that is currently still highly underdiagnosed particularly when it first manifests itself in adulthood.

Background: The Lewis (Le) blood group system, unlike most other blood groups, is not defined by antigens produced internally to the erythrocytes and their precursors but rather by glycan antigens adsorbed on to the erythrocyte membrane from the plasma. These oligosaccharides are synthesized by the two fucosyltransferases FUT2 and FUT3 mainly in epithelial cells of the digestive tract and transferred to the plasma. At their place of synthesis, some Lewis blood group carbohydrate antigen variants also seem to be involved in various gastrointestinal malignancies. However, relatively little is known about the transcriptional regulation of FUT2 and FUT3.

Summary: To address this question, we screened existing literature and additionally used in silico prediction tools to identify novel candidate regulators for FUT2 and FUT3 and combine these findings with already known data on their regulation. With this approach, we were able to describe a variety of transcription factors, RNA binding proteins and microRNAs, which increase FUT2 and FUT3 transcription and translation upon interaction.

Key messages: Understanding the regulation of FUT2 and FUT3 is crucial to fully understand the blood group system Lewis (ISBT 007 LE) phenotypes, to shed light on the role of the different Lewis antigens in various pathologies, and to identify potential new diagnostic targets for these diseases.

Introduction: ABO blood type changes after ABO-incompatible hematopoietic stem cell transplantation (HSCT). Most non-hematopoietic tissues retain the expression of the patient's own ABO antigens, which may adsorb from the plasma onto the donor's red blood cells (RBCs). Because of this phenomenon, a persistent patient's A and/or B antigen could be detected in the laboratory, despite 100% white cell donor chimerism. Adsorption of the patient's soluble ABO antigens on the newly formed RBCs complicates the interpretation of the patient's blood type and decision of transfusion therapy.

Case presentation: The first case report is a 6-year-old girl, A, D+, with T-cell acute lymphoblastic leukemia (ALL), transplanted with HLA-matched unrelated group O, D+ bone marrow. A second case report describes an 8-year-old girl, AB, D-, with ALL transplanted with an HLA-matched related group B, D+ bone marrow. The presence of persistent antigen A was registered in both patients more than 1 year after HSCT, despite complete donor chimerism.

Conclusion: The weak expression of ABO antigens on RBCs after HSCT should be examined in detail for proper planning of transfusion therapies.

Background: Erythrocyte alloantibodies and autoantibodies complicate transfusion. However, the prevalence of erythrocyte alloimmunization and autoimmunization has not been estimated in the Chinese pediatric population. Therefore, we investigated the prevalence of erythrocyte alloimmunization and autoimmunization in the Chinese pediatric population with the aim of developing a reasonable transfusion management policy in children from China.

Methods: This study included 30,603 pediatric inpatients who were admitted to three tertiary hospitals in central China from May 2020 to October 2022. Antibody screening was carried out with a three-cell panel by column agglutination technology, and samples with positive screening were analyzed for antibody specificity with a 16-cell identification panel. Clinical details of the patients were collected to identify associations with antibody formation.

Results: The alloimmunization rate was 0.55% (169/30,603), and the autoimmunization rate was 0.14% (43/30,603). Alloantibodies comprised 80.09% of the antibodies. The most frequent alloantibodies were anti-M (58.77%), anti-E (9.48%), and anti-P1 (4.27%). Autoantibodies comprised 19.91% of antibodies. Age (p = 0.000), sex (p = 0.016), geographical area (p = 0.000), ABO blood group (p = 0.008), and diagnosis (p = 0.000) were independent risk factors for antibody formation. The risk of antibody formation at the ages of 0-28 days and 1-3 months was zero (odds ratio = 0.000). The antibody distribution was significantly different by age (p = 0.000) and diagnosis (p = 0.000).

Conclusion: Repeat pre-transfusion testing for infants less than 4 months of age can be omitted for no risk of antibody formation. MNS system antibodies, especially anti-M, are prominent in younger children, and this decreases with age. Provision of extended phenotype-matched transfusion for Rh system antigens, especially antigen E, is necessary in children to control erythrocyte alloimmunization. The presence of antibodies with high evanescence rates in the pediatric population suggests the pressing need for nationwide shared transfusion records to avoid hemolytic transfusion reactions in children.

Background: The use of cell salvage and autologous blood transfusion is an important and widespread method of blood conservation during surgeries with expected high blood loss. The continuous autotransfusion device CATSmart^® (Fresenius Kabi, Germany) contains two new washing programs on the device called Flex wash 3 and Flex wash 5. To the best of our knowledge, there are no published clinical data regarding the performance of the two new washing programs.

Methods: In total, 69 patients undergoing cardiac or orthopedic surgery were included in this randomized, controlled, bicentric trial to validate the red cell separation process and washout quality of Flex wash 3 compared to Flex wash 5. After washing, the primary quality target was to determine hematocrit value, recovery rate, albumin, and total protein elimination rate in the packed red cells (PRCs). The secondary objective was to assess the elimination of heparin by measuring the factor anti-Xa activity by a 1- and 2-stage assay in PRC after washing.

Results: In the whole cohort of patients, hematocrit was 16.00% [9.15%; 21.30%] (median [Q1; Q3]) in the wound blood and 69.90% [51.10%; 80.90%] in the PRC resulting in a recovery rate of 63.92% [47.06%; 88.13%]. The albumin elimination rate was 98.77% [97.94%; 99.27%], and the total protein elimination rate was 98.85% [97.76%; 99.42%]. The heparin elimination rate was 99.95% [99.90%; 99.97%] in the 1-stage assay and 99.70% [99.41%; 99.87%] in the 2-stage assay. There was no difference between Flex wash 3 and Flex wash 5 washing procedure regarding the recovery rate 63.75% [46.64%; 78.65%] versus 67.89% [47.20%; 92.69%] (p = 0.85), albumin elimination rate 98.74% [97.67%; 99.27%] versus 98.78% [98.10%; 99.28%] (p = 0.97), protein elimination rate 98.79% [97.94%; 99.47%] versus 98.92% [97.58%; 99.42%] (p = 0.88), and anti-Xa elimination rate in the 1-stage assay 99.94% [99.79%; 99.97%] versus 99.95% [99.92%; 99.97%] (p = 0.24) and in 2-stage assay 99.66% [99.20%; 99.86%] versus 99.77% [99.47%; 99.90%] (p = 0.23).

Conclusions: The two new washing procedures, Flex wash 3 and Flex wash 5, enable sufficient and comparable red cell separation and washout quality of albumin, total protein, as well as heparin.

[This corrects the article DOI: 10.1159/000533624.].