Historically understudied and regarded as a mild type of sickle cell disease, HbSC can be associated with significant, progressive complications. Prospective studies are urgently needed to address treatment gaps for HbSC disease. Commentary on: Nelson et al. The clinical spectrum of HbSC sickle cell disease-not a benign condition. Br J Haematol 2024;205:653-663.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1111/bjh.19580 | DOI Listing |
Blood
January 2025
New York Blood Center, New York, New York, United States.
Babesiosis in sickle cell disease (SCD) is marked by severe anemia but the underlying red blood cell (RBC) rheological parameters remain largely undefined. Here, we describe altered RBC deformability from both primary (host RBC sickle hemoglobin mediated) and secondary changes (Babesia parasite infection mediated) to the RBC membrane using wild type AA, sickle trait AS and sickle SS RBCs. Our ektacytometry (LORRCA) analysis demonstrates that the changes in the host RBC bio-mechanical properties, pre- and post- Babesia infection, reside on a spectrum of severity, with wild type infected AA cells, despite showing a significant reduction of deformability under both shear and osmolarity gradients, exhibiting only a mild phenotype; compared to infected AS RBCs which show median changes in deformability and infected SS RBCs which exhibit the most dramatic impact of infection on cellular rheology, including an increase in Point of Sickling values.
View Article and Find Full Text PDFPak J Med Sci
January 2025
Khalid Khalil Security Forces Hospital Makkah, Makkah, Saudi Arabia.
Objective: To observe the fetomaternal outcome of therapeutic versus prophylactic blood transfusions in patients with sickle cell disease (SCD) during pregnancy.
Method: This single-center retrospective observational study was conducted on consecutive pregnant women with SCD between January 2018 and December 2020. All the pregnant women with SCD were included in this study.
Non-myeloablative hematopoietic cell transplantation (HCT) is a curative option for individuals with sickle cell disease (SCD). Our traditional goal with this approach has been to achieve a state of mixed donor/recipient chimerism. Recently, we reported an increased risk of hematologic malignancies (HMs) in adults with SCD following graft failure or mixed chimerism.
View Article and Find Full Text PDFCureus
December 2024
Internal Medicine, King Fahad Hospital Hofuf, Hofuf, SAU.
Sickle cell anemia (SCA) is one of the known hemoglobinopathies that result in red blood cell (RBC) destruction, among other complications. There are factors that make SCA an environment for autoimmune disease (AID). They include chronic inflammation, immune-mediated processes involved in SCA complications, and susceptibility to infections.
View Article and Find Full Text PDFReumatologia
December 2024
Department of Medicine, Lagos State University Teaching Hospital, Ikeja, Nigeria.
Introduction: Systemic lupus erythematosus (SLE) and sickle cell disease (SCD) are distinct multisystemic diseases that commonly affect blacks. There are few reports of their co-existence in Western literature and a paucity of reports in Sub-Saharan Africa. Their co-existence is associated with diagnostic delay and treatment dilemmas.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!