Increased Vasoocclusive Crisis in “O” Blood Group Sickle Cell Disease Patients: Association With Underlying Thrombospondin Levels.

Huneini, Mohamed Al; Alkindi, Salam; Panjwani, V.; Falahi, K. Al; Balushi, B. Al; Gravell, David; Ho, Chester; Krishnamoorthy, R. V.; Pathare, Anil

doi:10.4084/mjhid.2017.028

Cited by 4 publications

(4 citation statements)

References 54 publications

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“…In the current evaluable cohort of 44 patients with sickle cell disease (SCD) (Table 1), the mean SF (±SD) reduced marginally from a baseline value of 4,311 (4,030) ng/ml to 4,230 (3,059) ng/ml. Mean LIC (±SD) by Liver T2 * MRI dropped marginally from 12 (9) to 10.3 (7) mg/gm dry wt. Mean Cardiac T2 * MRI (±SD) improved marginally from 36.8 (17) to 39.5 (6) ms.…”

Section: Resultsmentioning

confidence: 87%

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Iron Overload in Patients With Heavily Transfused Sickle Cell Disease—Correlation of Serum Ferritin With Cardiac T2* MRI (CMRTools), Liver T2* MRI, and R2-MRI (Ferriscan®)

et al. 2021

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The treatment of sickle cell disease (SCD) is mainly supportive, except for a minority, who receive bone marrow transplantation (BMT). Serum ferritin (SF) is routinely available but is notoriously unreliable as a tool for iron-overload assessment since it is an acute-phase reactant. Although blood transfusion is one of the most effective ways to deal with specific acute and chronic complications of SCD, this strategy is often associated with alloimmunization, iron overload, and hemolytic reactions. This study, thus, aims to evaluate iron overload in patients with SCD on chronic blood transfusions and specifically, correlate SF with the current standard of care of iron-overload assessment using MRI-based imaging techniques. Amongst a historic cohort of 58 chronically transfused patients with SCD, we were able to evaluate 44 patients who are currently alive and had multiple follow-up testing. Their mean age (±SD) was 35 (9) years and comprised of 68.2% of women. The studied iron-overload parameters included cardiac T2* MRI, liver iron concentration (LIC) by Liver T2* MRI, and serial SF levels. Additionally, in a smaller cohort, we also studied LIC by FerriScan© R2-MRI. Chronic blood transfusions were necessary for severe vaso-occlusive crisis (VOC) (38.6%), severe symptomatic anemia (38.6%), past history of stroke (15.9%), and recurrent acute chest syndrome (6.9%). About 14 (24%) patients among the original cohort died following SCD-related complications. Among the patients currently receiving chelation, 26 (96%) are on Deferasirox (DFX) [Jadenu® (24) or Exjade® (2)], with good compliance and tolerance. However, one patient is still receiving IV deferoxamine (DFO), in view of the significantly high systemic iron burden. In this evaluable cohort of 44 patients, the mean SF (±SD) reduced marginally from 4,311 to 4,230 ng/ml, mean Liver T2* MRI dropped from 12 to 10.3 mg/gm dry weight, while the mean cardiac T2*MRI improved from 36.8 to 39.5 ms. There was a mild to moderate correlation between the baseline and final values of SF ng/ml, r = 0.33, p = 0.01; Cardiac T2* MRI ms, r = 0.3, p = 0.02 and Liver T2* MRI mg/kg dry weight, r = 0.6, p < 0.001. Overall, there was a positive correlation between SF and Liver T2* MRI (Pearson's r = 0.78, p < 0.001). Cardiac T2*MRI increased with the decreasing SF concentration, showing a negative correlation which was statistically significant (Pearson's r = −0.6, p < 0.001). Furthermore, there was an excellent correlation between SF ng/ml and LIC by FerriScan© R2-MRI mg/g or mmol/kg (Spearmen's rho = −0.723, p < 0.008) in a small subset of patients (n = 14) who underwent the procedure. In conclusion, our study demonstrated a good correlation between serial SF and LIC by either Liver MRI T2* or by FerriScan© R2-MRI, even though SF is an acute-phase reactant. It also confirms the cardiac sparing effect in patients with SCD, even with the significant transfusion-related iron burden. About 14 (24%) patients of the original cohort died over the past 15 years, indicative of a negative impact of iron overload on disease morbidity and mortality.

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Section: Resultsmentioning

confidence: 87%

“…A yearly increase as the result of newborn cases is about 120-150 (3). Thus, SCD is a major public health problem in the Sultanate of Oman, with a high rate of morbidity and mortality (4)(5)(6)(7)(8)(9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

Iron Overload in Patients With Heavily Transfused Sickle Cell Disease—Correlation of Serum Ferritin With Cardiac T2* MRI (CMRTools), Liver T2* MRI, and R2-MRI (Ferriscan®)

et al. 2021

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“… 14 In the ABO blood group system, individuals with group O blood are more prone to vaso-occlusive crises associated with sickle cell disease (SCD). 16 However, the sickle hemoglobin of SCD combined with the O- blood group also confers some resistance to Plasmodium falciparum infection. 17 Additionally, group O blood has also been found to confer resistance toward developing cardiovascular disease and/or type-2 diabetes.…”

Section: Introductionmentioning

confidence: 99%

Using whole-genome sequencing to characterize clinically significant blood groups among healthy older Australians

et al. 2022

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There have been no comprehensive studies of a full range of blood group polymorphisms within the Australian population. The problem is compounded by the absence of any databases carrying genomic information on chronically transfused patients and low frequency blood group antigens in Australia. Here, we use RBCeq, a web server-based blood group genotyping software, to identify unique blood group variants among Australians and compare the variation detected versus global data. Whole genome sequencing data was analysed for 2796 healthy older Australians from the Medical Genome Reference Bank and compared with data from 1000G phase 3 (1KGP3) databases comprising 661 African, 347 American, 503 European, 504 East Asian, and 489 South Asian participants. There were 661 rare variants detected in this Australian sample population, including nine variants that had clinical associations. Notably, we identified 80 variants that were computationally predicted to be novel and deleterious. No clinically significant rare or novel variants were found associated with the genetically complex ABO blood group system. For the Rh blood group system, two novel and 15 rare variants were found. Our detailed blood group profiling results provide a starting point for the creation of an Australian blood group variant database.

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“…Plasma TSP which is released from activated platelets attaches to the quiescent and activated ECs that is therefore believed to promote adhesion of SS-RBCs to the endothelium in vivo. Additionally, TSP attaches to endothelial integrin complex αvβ3 to sulfated glycans on the sickled cells surfaces [27, 28]. ICAM-1, VCAM-1, P-selectin and E-selectin and the procoagulant molecule tissue factor are all expressed on activated endothelium and play pivotal parts in the WBCs oriented locomotion and the promotion of thrombosis within the inflamed vascular system.…”

Section: Introductionmentioning

confidence: 99%

Role of some members of chemokine/cytokine network in the pathogenesis of thalassemia and sickle cell hemoglobinopathies: a mini review

et al. 2019

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The word of hemoglobinopathy is described for an array of disorders that affecting hemoglobin (Hb) functions. Hb is a molecule with 68 kDa molecular weight, serving as oxygen carrying metalloprotein. Hemoglobinopathy includes a wide range of Hb structural deficits varying from thalassemia to sickle cell disease. Cyto-chemokine network members are pivotally involved in the pathogenesis of hemoglobinopathies, however, the exact role of these mediators in the development of these disorders yet to be well addressed. Cytokines and chemokines are generated by inflamed endothelial cells that promote the expression of their respected receptors and further activate NF-κβ, recruit red blood cells (RBCs) and white blood cells (WBCs) toward the inflamed endothelium. Therefore, due to critical roles played by the cyto-chemokine network in several aspects of hemoglobinopathies pathophysiology including apoptosis of endothelial cells, RBC, WBC and etc.…, in the present review, we focused on the critical parts played by this network in the pathogenesis of hemoglobinopathies.

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Increased Vasoocclusive Crisis in “O” Blood Group Sickle Cell Disease Patients: Association With Underlying Thrombospondin Levels.

Cited by 4 publications

References 54 publications

Iron Overload in Patients With Heavily Transfused Sickle Cell Disease—Correlation of Serum Ferritin With Cardiac T2* MRI (CMRTools), Liver T2* MRI, and R2-MRI (Ferriscan®)

Iron Overload in Patients With Heavily Transfused Sickle Cell Disease—Correlation of Serum Ferritin With Cardiac T2* MRI (CMRTools), Liver T2* MRI, and R2-MRI (Ferriscan®)

Using whole-genome sequencing to characterize clinically significant blood groups among healthy older Australians

Role of some members of chemokine/cytokine network in the pathogenesis of thalassemia and sickle cell hemoglobinopathies: a mini review

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