bims-sicedi Biomed News
on Sickle cell disease
Issue of 2025–12–28
seven papers selected by
João Conrado Khouri dos Santos, Universidade de São Paulo



  1. Front Immunol. 2025 ;16 1699306
       Background: Monocytes can interact with erythroid cells and contribute to macrophage pools under anemic stress, thereby supporting the erythropoietic niche. In sickle cell anemia (SCA), extensive hemolysis and ineffective erythropoiesis may expose circulating monocytes to abnormal red blood cells (RBCs), potentially altering their phenotype and function to accommodate the higher erythropoietic response and iron demand.
    Methods and results: We characterized circulating monocytes from SCA patients at steady state using flow cytometry and in vitro phagocytosis assays. Intracellular RBC material was detected in all circulating monocyte subsets from SCA patients, indicating active erythrophagocytosis. Mechanistically, SCA monocytes displayed upregulated VCAM-1 and reduced SIRP-α expression, favoring RBC binding and internalization even when CD47 expression on RBCs was preserved. Following RBC engulfment, monocytes upregulated heme oxygenase-1 (HO-1) and ferroportin (FPN), consistent with enhanced heme degradation and iron export, and expressed higher levels of CD206, suggesting a regulatory phenotype. In vitro assays confirmed that both sickle RBCs and SCA monocytes synergistically promoted erythrophagocytosis.
    Conclusion: Circulating monocytes from SCA patients undergo phenotypic and functional reprogramming upon interaction with sickled RBCs, acquiring features reminiscent of erythroblastic island macrophages. These findings highlight a previously underappreciated role for monocytes in RBC clearance, heme metabolism, and iron recycling in SCA, with potential implications for inflammation and disease progression.
    Keywords:  erythroblastic island; hemolytic anemia; iron metabolism; macrophages; sickle cell disease
    DOI:  https://doi.org/10.3389/fimmu.2025.1699306
  2. Exp Biol Med (Maywood). 2025 ;250 10806
      Sickle cell disease (SCD) is a severe inherited hemoglobinopathy with limited curative treatment options. In December 2023, the U.S. FDA approved two autologous gene therapies, lovo-cel (bluebird bio) and exa-cel (Vertex/CRISPR Therapeutics), offering potentially transformative outcomes. We performed a comparative analysis of these therapies based on published clinical trial design, patient eligibility, manufacturing requirements, and reported efficacy and safety outcomes. Overall, participants treated with lovo-cel had more severe baseline disease, reflected by a higher median rate of vaso-occlusive events (VOEs), despite the use of a more stringent VOE definition. Mobilization of hematopoietic stem cells (HSCs) with single-agent plerixafor proved challenging in both trials, with most participants requiring multiple mobilization and apheresis cycles. A greater proportion of exa-cel participants required three or more apheresis procedures, driven by higher CD34+ cell dose targets needed to compensate for CRISPR-associated HSC loss. Both therapies demonstrated greater than 90% resolution of severe VOEs, with near-complete resolution in pediatric participants. A small subset of participants experienced VOEs post-treatment, including events occurring beyond the primary efficacy assessment period. Notably, no recurrent strokes were reported among lovo-cel treated participants with a history of overt stroke. Both therapies provide durable, clinically meaningful benefit and represent a major advancement in SCD management. However, differences in trial populations, cell collection logistics, and manufacturing have important implications for real-world applications. Continued long-term follow-up and the establishment of standardized post-treatment registries will be critical to fully assess durability, monitor late effects, and inform patient selection.
    Keywords:  autologous transplantation; exal-cel; gene therapy; lovo-cel; sickle cell disease
    DOI:  https://doi.org/10.3389/ebm.2025.10806
  3. Am J Nurs. 2026 Jan 01. 126(1): 26-33
       ABSTRACT: Sickle cell disease (SCD) is an inherited red blood cell disorder that affects an estimated 70,000 to 100,000 people in the United States, most of whom are of African descent. SCD causes acute and chronic pain and widespread end-organ damage, resulting in acute and chronic complications, high morbidity, and early death. Advances in research and treatment have improved patient outcomes for people living with SCD. Over the past 10 years, several new medications and curative therapies have been approved by the U.S. Food and Drug Administration for people with SCD, and more medications are in the pipeline. This review includes historical perspectives, current treatment options, and future directions for this population. With the ongoing rapid changes in SCD care, nurses are well positioned to play a role in patient education, research, advocacy, and the implementation of these emerging therapies to advance the care of those affected by SCD.
    Keywords:  access to care; clinical updates; curative therapies; sickle cell disease
    DOI:  https://doi.org/10.1097/AJN.0000000000000216
  4. Am J Hematol. 2025 Dec 20.
      Sickle cell anemia (SCA) leads to reduced physical functioning and cardiopulmonary fitness. Prior studies suggest that airway hyperresponsiveness to bronchoprovocation testing is common in SCA, but the prevalence of exercise-induced bronchospasm (EIB) is understudied. We hypothesized that EIB is more common in children with SCA than in controls. Non-asthmatic subjects 10-21 years old with SCA and race-matched controls underwent (1) maximal Cardiopulmonary Exercise Testing (CPET) by cycle ergometry and (2) a Controlled Intensity Interval Test (CIIT) consisting of eight bouts of constant workload cycling, randomized to 50% (moderate) or 70% (vigorous) of peak workload. Spirometry was performed pre/post CPET and CIIT. Multivariable logistic regression models tested associations between SCA status and EIB in response to CPET and CIIT. Compared to controls, subjects with SCA demonstrated lower hemoglobin, reduced baseline spirometry values, and decreased CPET maximal workload. Baseline lower airway obstruction and completion rates for CPET and CIIT were similar between groups. No adverse events occurred. The percentage of participants who met criteria for EIB did not differ between subjects and controls after CPET (21% vs. 26%, p = 0.537) or CIIT (32% vs. 17%, p = 0.126). In adjusted models, SCA status was not associated with EIB after CPET or CIIT. EIB was not more common in subjects with SCA versus controls after maximal CPET or submaximal exercise challenge of longer duration. Further research is needed to inform the development of exercise guidelines and to better understand the effects of exercise on airway dynamics in SCA. Trial Registration: ClinicalTrials.gov identifier: NCT03653676.
    Keywords:  airway responsiveness; exercise challenge; exercise induced bronchospasm; sickle cell anemia
    DOI:  https://doi.org/10.1002/ajh.70170
  5. Blood Glob Hematol. 2025 Jun;pii: 100001. [Epub ahead of print]1(1):
      Sickle cell anemia (SCA) is an inherited hemolytic anemia that causes stroke in children. Transcranial Doppler ultrasound (TCD) of the intracerebral arteries quantifies primary stroke risk. Chronic blood transfusions reduce risk but are not always feasible in lower resource settings. Hydroxyurea decreases TCD velocities in SCA, but the effect size and duration are unclear. We conducted a systematic review to understand hydroxyurea's effectiveness in children. We searched 5 major medical databases (CINAHL, EMBASE, Trip Medical Database, Scopus, and PubMed) and identified prospective clinical trials that enrolled children with SCA, performed TCD screening before hydroxyurea treatment, and collected serial measurements of TCD velocities and stroke incidence during hydroxyurea treatment. Citations were screened for inclusion, eligible citations selected, and data extracted. A total of 104 reports describing 13 clinical trials with 592 participants were included in the review. Hydroxyurea decreased TCD with a mean decline of -30 cm/s (95% confidence interval, -41 to -19) over 0.5 to 2.6 years of therapy. The TCD velocity normalized in most children. Stroke was reported in 3 trials, only occurred in those with persistent abnormal TCD values (>200 cm/s), and had lower incidence than expected (0.52-1.92 per 100 patient-years). Hydroxyurea is an effective strategy for reducing TCD velocities and stroke risk in children with SCA and is a feasible alternative when transfusions are unavailable, especially in resource-limited settings. Additional research is needed to clarify the effect on stroke incidence and optimal dosing strategies for durable treatment effect, long-term safety, and comprehensive benefits in diverse health care settings.
    DOI:  https://doi.org/10.1016/j.bglo.2025.100001