S-glutathionyl hemoglobin is a proposed biomarker of oxidative stress but has not been measured in sickle cell disease patients. Unlike the S-glutathionyl adduct of normal adult hemoglobin, S-glutathionyl sickle hemoglobin (HbSSG) cannot be directly measured by capillary isoelectric focusing, because it coelutes with fetal hemoglobin (HbF). This suggests that HbF, measured in sickle cell patients with or without hydroxyurea therapy, might contain endogenous HbSSG. As S-glutathionyl hemoglobin can form during sample storage, HbSSG could falsely elevate HbF levels in stored samples. We measured HbSSG based on the quantitative difference in the heterogeneous HbF/HbSSG peak before and after hemolysates were treated with dithiothreitol. Paired t tests showed that dithiothreitol reduced HbF/HbSSG in blood from pediatric sickle cell patients (n=25, mean decrease+/-SD=1.0%+/-0.6, P<0.05) but not in normal infants (n=25). Higher HbF levels in hydroxyurea-treated patients (n=5) were not attributable to HbSSG. HbSSG increased significantly within 1 day in samples stored at -20 degrees C but was unchanged in samples stored 60 days at-70 degrees C. We conclude that blood from sickle cell patients contained up to 2.2% HbSSG, and that endogenous HbSSG is a minor interferent in the measurement of HbF in fresh blood but a major interferent in improperly stored samples.
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http://dx.doi.org/10.1097/MPH.0b013e3181bc9c9b | DOI Listing |
J Pediatr Hematol Oncol
January 2025
Medical Oncology, Haemato-Oncology, BMT, Sarvodaya Hospital, Faridabad, Haryana, India.
Background And Objective: Sickle cell disease (SCD) is a genetically inherited disorder that is associated with morbidity and mortality.
Methods: This cross-sectional study was conducted on patients diagnosed with SCD to evaluate the knowledge, attitude, and practice of patients/guardians using a pretested questionnaire.
Results And Discussion: Of the 111 participants, 56 (50.
Eur J Haematol
January 2025
Division of Hematology and Medical Oncology, Oregon Health & Science University, Portland, Oregon, USA.
Hematologic complications are common in pregnancy and can significantly impact both maternal and fetal health. Recognizing and treating these complications can be challenging due to the limited evidence available to guide clinical consultants. Iron deficiency anemia is the most prevalent hematologic issue in pregnancy and often occurs due to increased maternal blood volume and the nutritional demands of the growing fetus.
View Article and Find Full Text PDFClin Exp Med
January 2025
Department of Zoology, Faculty of Science, Ain Shams University, Abbassia, Cairo, 11566, Egypt.
The demand for sensitive, rapid, and affordable diagnostic techniques has surged, particularly following the COVID-19 pandemic, driving the development of CRISPR-based diagnostic tools that utilize Cas effector proteins (such as Cas9, Cas12, and Cas13) as viable alternatives to traditional nucleic acid-based detection methods. These CRISPR systems, often integrated with biosensing and amplification technologies, provide precise, rapid, and portable diagnostics, making on-site testing without the need for extensive infrastructure feasible, especially in underserved or rural areas. In contrast, traditional diagnostic methods, while still essential, are often limited by the need for costly equipment and skilled operators, restricting their accessibility.
View Article and Find Full Text PDFPediatr Blood Cancer
January 2025
Blood and Marrow Transplant/Cellular Therapy Program, Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
With advances in conditioning strategies and graft-versus-host disease (GvHD) prevention, hematopoietic stem cell transplantation (HSCT) is a safe, curative treatment option for pediatric patients with sickle cell disease (SCD). However, donor options have been limited in non-myeloablative matched sibling donor (MSD) setting by excluding recipients with major ABO blood group incompatible donors due to concern of the risk of significant complications such as pure red cell aplasia (PRCA). We present three cases of successful HSCT with major ABO incompatibility with their donors, and discuss strategies to safely expand the donor pool to include these donors.
View Article and Find Full Text PDFInt J Nurs Stud
December 2024
Service de Médecine Interne, Centre National de Référence des Syndromes Drépanocytaires Majeurs de l'Adulte, AP-HP, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, 20 rue Leblanc, F-75908 Paris cedex 15, France; Service d'aval des urgences, hopital Henri-Mondor Assistance Publique-Hôpitaux de Paris, 1 rue Gustave Eiffeil, 94000 Créteil, France; Université Paris Cité, Paris, France. Electronic address:
Background: Intravenous (IV) access is often required for the treatment of vaso-occlusive crises in patients with sickle cell disease, but can be particularly challenging due to recurrent venous damage. The AccuVein® device, uses near-infrared light technology to visualise veins for easier venepuncture.
Methods: A randomised, controlled trial of the efficacy of the AccuVeinAV400® device in the replacement of peripheral venous lines during a vaso-occlusive crisis was conducted at two centres in France.
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