Haptoglobin Genotypes Associated with Vaso-Occlusive Crisis in Sickle Cell Anemia Patients of Eastern India

Meher, Satyabrata; Mohanty, P.; Patel, Siris; Das, Kunal; Sahoo, Sarmila; Dehury, Snehadhini; Mohapatra, Manoranjan; Jit, Bimal Prasad; Das, Padmalaya; Dash, Bisnu Prasad

doi:10.1080/03630269.2020.1801459

Cited by 10 publications

(14 citation statements)

References 34 publications

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Section: Resultsmentioning

confidence: 96%

“…8,26 The association of the HP genotypes with circulating HP concentrations in patients with SCD is less clear. One SCD cohort demonstrated that the HP 1-1 genotype was associated with higher HP concentrations than HP 2-1 or 2-2, 16 whereas another study did not find any difference. 27 We observed that the HP 1 allele is associated with higher HP and lower cell-free hemoglobin concentrations at steady state as well as during VOE.…”

Section: Hp Genotypementioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

“…10,11 Furthermore, some non-SCD studies have demonstrated that the HP 1-1 genotype is protective from kidney injury and cardiovascular disease, 7,12 whereas others indicate that this genotype is a risk factor for neurologic or cardiovascular complications compared with the HP 2-2 genotype. 13,14 Studies in patients with SCD have also shown mixed results, with some demonstrating that the HP 1 allele is associated with fewer SCD-related complications 15,16 and others not reporting differences by the HP genotype. 17,18 We conducted a longitudinal study to determine whether the (1) HP genotype is associated with HP and cell-free hemoglobin concentrations at steady state, VOE, and acute chest syndrome; and (2) HP 1 allele is protective against developing multiorgan failure during an episode of acute chest syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Haptoglobin 1 allele predicts higher serum haptoglobin concentration and lower multiorgan failure risk in sickle cell disease

et al. 2022

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Haptoglobin (HP) is an acute phase protein and the main scavenger of cell-free hemoglobin. When haptoglobin is depleted, as observed in hemolytic conditions such as sickle cell disease (SCD), cell-free hemoglobin can lead to acute organ damage. The impact of the HP 1-1, 2-1, and 2-2 isoforms on HP and cell-free hemoglobin concentrations and SCD-related complications is unclear. In a longitudinal cohort of SCD patients, the HP 1 allele was associated with higher HP and lower cell-free hemoglobin concentrations at a routine clinic visit as well as during hospitalization for a vaso-occlusive episode (VOE) or acute chest syndrome. With a median follow up of 6.8 years, acute chest syndrome occurred in 42% (n=163) and multiorgan failure in 14% (n=53) of 391 SCD patients with a minimum follow-up of six months. The HP 1 allele was independently associated with lower risk of developing multiorgan failure during acute chest syndrome (additive model HR 0.5; P<0.001). Future studies assessing the regulation of HP concentrations and ability to bind cell-free hemoglobin according to HP genotype may help to identify SCD patients at high risk for multiorgan failure and to guide interventions, such as rapid initiation of exchange transfusion or HP replacement therapy.

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Section: Resultsmentioning

confidence: 96%

Section: Hp Genotypementioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Haptoglobin 1 allele predicts higher serum haptoglobin concentration and lower multiorgan failure risk in sickle cell disease

et al. 2022

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“…The population of the included studies consisted of people of all ages, and both sexes. Of the 33 included studies, 14 were from Africa, 7,20–30 4 from Europe, 8,31–33 8 from South America, 6,11,34–39 4 from America, 40–43 3 in Asia 1,44–46 . The included studies evaluated hemoglobin, ferritin, serum iron, C‐reactive protein (CRP) and transferrin chiefly.…”

Section: Resultsmentioning

confidence: 99%

Homozygote drepanocytosis: Ferric status and inflammation in world and Africa: Review article and meta analysis

Tuono De Manfouo,

Louokdom,

Chetcha

et al. 2023

Health Science Reports

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Background and AimsMajor sickle cell syndromes are subjected to a high frequency of hemolysis, infections, oxidative stress, and vasooclusive crises which promote inflammation and iron balance disorders. We aimed to systematically review and analyze the studies in this patients addressing in general, and Africa in particular.MethodsThe systematic review of published articles in the Pubmed and Google Scholar databases was carried out according to the recommendations of the PRISMA model. The case‐control articles have been included. The data extracted from the articles were analyzed using statistical software R. The standardized mean difference (SMD) was used to assess the extent of the disease on the different variables studied.ResultsAt the end, 128 articles were obtained; but only 33 were elligible for meta‐analysis. A SMD of −1.79 was obtained for hemoglobin between the sickle cell patients and the controls due to the deviation from the overall mean hemoglobin in the cases (8 ± 2 g/dL) and in controls (13 ± 3 g/dL). Sickle cell disease showed a significant extent on ferritin [SMD = 2.61; (95% confidence interval, CI: 2.39–2.83); (p < 0.01)] compared to non‐sickle cell patients thus describing a higher risk for sickle cell sufferer to have ferritin disorders. The included studies also described the influence of sickle cell anemia on serum iron [SMD = 1.52; (95% CI: 1.32–1.76); (p < 0.01)] compared to normal subjects. The high risk of inflammation has been described as higher in sickle cell patients [SMD = 0.38; (95% CI: 0.25–0.50)], reflecting the moderate extent of sickle cell disease on inflammation.ConclusionPatients with major sickle cell syndrome in inflammation have a higher risk of iron profile disorders compared to the normal population. Further studies are needed to explore mechanisms for preventing the deleterious effects of iron from this hemolysis, for example haptoglobin genotyping.

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Haptoglobin gene polymorphism and iron profile in sickle cell disease patients with inflammation in Yaounde, Cameroon

Tuono,

Simo,

Nya

et al. 2023

Molec Gen & Gen Med

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BackgroundMajor sickle cell syndromes are the most common hemoglobinopathy in the world. The sickle cell patients are subjected to several factors causing inflammation, and the genetic identification of each individual allows to focus the possibility of allelic variations influence of a specific gene and then the polymorphism. This study aims at determining the distribution of HP gene (OMIM#140100) and their involvement on hematological parameters and the iron profile in the sickle cell patients presenting an inflammation condition during major sickle cell syndromes in Cameroun.MethodsA case–control analytical study has been conducted over a period of 6 months. Cases consisting of sickle cell patients in a situation of inflammation and control of non‐inflamed sickle cell patients. The patients presenting major sickle cell syndromes, interned and/or followed at the Hematology Department of the Regional Hospital of Bafoussam and the Central Hospital of Yaoundé have been recruited. HP genotyping was carried out at the Laboratory for Public Health Research Biotechnologies (LAPHER‐Biotech) in Yaoundé using allele‐specific PCR. Also, inflammatory, hematological parameters and martial assessment were explored by standard methods. Statistical analysis of the data was performed using the statistical tool R version 4.1.1. The comparison of proportions of alleles was made with the chi‐square test, and the Wilcoxon test was used to compare the median between different groups using the statistical tool R version 4.1.1.ResultsWe analyzed the samples of 149 patients. The HP polymorphism describes a significant frequency of the “1F” allele (69.8%) followed by the “2” allele (46.31%). In addition, 80 patients (53.69%), 48 (32.21%), and 21 (14.09%) presented the genotype HP 1‐1, HP 2‐1, and HP 2‐2, respectively. And eighty‐one percent (81%) patients with genotype HP 2‐2 showed a significant higher relative frequency of thrombocytosis compared with the genotype HP 1‐1 and HP 2‐1, respectively (51.2% and 68.8%, p = 0.087). The proportion of inflammation in the HP 2‐2 group was higher (57.1%) compared with the other groups (respectively 42.5% and 35.4% in the HP 1‐1 and HP 2‐1 groups). Furthermore, the median CRP was significantly higher in the HP 2‐2 group compared with the other groups (p = 0.039). Moreover, the entire population of the HP 2‐2 group showed an elevation of ferritin and IL6 unlike the HP 1‐1 and HP 2‐1 groups.ConclusionThis study demonstrates a higher frequency of genotype HP 1‐1 followed by the HP 2‐2 genotype in patients with major sickle cell syndromes. However, a larger proportion of patients with genotype HP 2‐2 are associated with hematological profile disorders, inflammation, and dysregulation of iron metabolism. Then, the haptoglobin polymorphism contributes to the severity of major sickle cell syndromes.

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Haptoglobin Genotypes Associated with Vaso-Occlusive Crisis in Sickle Cell Anemia Patients of Eastern India

Cited by 10 publications

References 34 publications

Haptoglobin 1 allele predicts higher serum haptoglobin concentration and lower multiorgan failure risk in sickle cell disease

Haptoglobin 1 allele predicts higher serum haptoglobin concentration and lower multiorgan failure risk in sickle cell disease

Homozygote drepanocytosis: Ferric status and inflammation in world and Africa: Review article and meta analysis

Haptoglobin gene polymorphism and iron profile in sickle cell disease patients with inflammation in Yaounde, Cameroon

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