Epidemiological, clinical, and severity characterization of sickle cell disease in a population from the Brazilian Amazon

Cesar, Purim; Dhyani, Anamika; Schwade, Luiz Augusto; Acordi, Pollyana; Albuquerque, Cinthia Xerez; Nina, Rejane; Paula, Erich Vinícius De; Fraiji, Nelson Abrahim

doi:10.1016/j.hemonc.2019.04.002

Cited by 7 publications

(4 citation statements)

References 22 publications

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“…The choice of SCA subgroups was based on the clinical importance of hemolysis (reticulocytes), hemostasis, and inflammation (platelets) and clinical complications inherent to the physiopathology of sickle cell anemia. The association of reticulocytosis and thrombocytosis (platelet count above 400 × 10 6 /mm 3 ) in the clinical severity of this disease was shown in previous studies [28]. The categorizations of these subgroups were done by GraphPad Prism version 5.0 software (San Diego, CA, USA) using descriptive analysis establishing the minimum 25% percentile, the median, and the maximum 75% percentile.…”

Section: Introductionmentioning

confidence: 90%

Sickle Cell Anemia Patients Display an Intricate Cellular and Serum Biomarker Network Highlighted by TCD4+CD69+ Lymphocytes, IL-17/MIP-1β, IL-12/VEGF, and IL-10/IP-10 Axis

Garcia

Júnior

Soares

et al. 2020

Journal of Immunology Research

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Background. Sickle cell anemia (SCA) is associated with a chronic proinflammatory state characterized by elevated leukocyte count, mortality from severe recurrent infections, and subsequent vasoocclusive complications with leukocyte adhesion to the endothelium and increased plasma levels of inflammatory cytokines. The immune system has a close connection with morbidity in SCA, but further studies are needed to uncover the involvement of innate and adaptive immunities in modulating the SCA physiopathology. We performed measurements of the frequency of innate and adaptive immunity cells, cytokines, chemokines, and growth factors and immunophenotyping of Toll-like receptor and adhesion molecule expression in the blood of SCA patients and healthy donors to evaluate the different profiles of these biomarkers, the relationship among them, and their correlation to laboratory records and death risk. Material and Methods. Immunophenotyping of cells, Toll-like receptors, and adhesion molecules were performed from peripheral blood samples of SCA patients and healthy donors by flow cytometry and cytokine/chemokine/growth factor measurement by the Luminex technique performed from the serum of the same subjects. Results. Cells of adaptive immunity such as IL-12, IL-17, and IL-10 cytokines; IL-8, IP-10, MIP-1α, MIP-1β, and RANTES chemokines; and VEGF, FGF-basic, and GM-CSF growth factors were higher in SCA patients than healthy donors regardless of any laboratorial and clinical condition. However, high death risk appears to have relevant biomarkers. Conclusion. In the SCA pathophysiology at steady state, there is a broad immunological biomarker crosstalk highlighted by TCD4+CD69+ lymphocytes, IL-12 and IL-17 inflammatory and IL-10 regulatory cytokines, MIP-1α, MIP-1β, and IP-10 chemokines, and VEGF growth factor. High expression of TLR2 in monocytes and VLA-4 in TCD8+ lymphocytes and high levels of MIP-1β and RANTES appear to be relevant in high death risk conditions. The high reticulocytosis and high death risk conditions present common correlations, and there seems to be a balance by the Th2 profile.

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Section: Introductionmentioning

confidence: 90%

Sickle Cell Anemia Patients Display an Intricate Cellular and Serum Biomarker Network Highlighted by TCD4+CD69+ Lymphocytes, IL-17/MIP-1β, IL-12/VEGF, and IL-10/IP-10 Axis

Garcia

Júnior

Soares

et al. 2020

Journal of Immunology Research

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“…Furthermore, the patients' follow-up period was less than 20 years in a little less than half of the cases. Other Brazilian states such as Amazonas 25 -North region -and Paraíba 26 -Northeast region -also present similar data concerning gender and age group, follow-up period, and age at diagnosis.…”

Section: Discussionmentioning

confidence: 52%

“…This is due to the territorial distance separating them from the specialized centers for treatment and the logistical difficulties the patients face. 25,28,35 In addition to the vasoclusive conditions, the number of hemotransfusions and the hemoglobin levels report an important event in the course of the disease: anemia. A common condition observed in patients with SCA, anemia, occurs mainly due to the reduced half-life of the red blood cells.…”

Section: Discussionmentioning

confidence: 99%

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018

Pompeo¹,

Júnior²,

Cardoso³

et al. 2022

IJGM

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To analyze the clinical-epidemiological characteristics and mortality in patients with sickle-cell anemia (SCA). Patients and Methods: A cohort study with retrospective data, conducted in two reference hospitals for SCA treatment from January 1980 to December 2018, recorded in two reference services. With a 5% significance level, the Chi-Square and Student's t-tests were employed in the inferential statistical analysis. Results: A total of 128 patients with SCA were studied. Diagnosis up to the fifth day of life was made in 10 patients. There were 19 deaths, of which 12 (63.2%) were female, and the average age at death was 27.05 (± 14.78) years. The leading causes of death were septic shock and cardiogenic shock. The use of invasive medical devices was considered a risk factor for death (RR=2.63; 95% CI=1.16-5.96; p=0.018), and monitoring time up to 20 years presented a 31% reduction in the risk of dying (RR=0.31; 95% CI=0.12-0.82; p=0.011) when compared to the monitoring of more than 20 years. Conclusion: These findings are to be considered in the treatment of patients with SCA, mainly regarding early diagnosis and access to the treatment immediately afterward, since they are fundamental in improving survival and reducing severe complications.

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“…These theories support the adaption of human entities to microbial enemies, related to the geographical distribution of the mutated haemoglobin alleles [40]. Epidemiological reports from various studies have been focused on the relation between the Plasmodium falciparum parasite and the SCD's epidemiology [41]. Despite the overwhelming epidemiological sector of SCD due to the malaria existence, the study of Wambua et al investigated the importance of the major haemoglobinopathies to the protection against the complications of the parasite infection.…”

Section: Discussionmentioning

confidence: 99%

The Anti-Sickling Properties of Medicinal Plants, Insights in Botanical Medicine*

Krasias¹

2021

AJMB

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Epidemiological, clinical, and severity characterization of sickle cell disease in a population from the Brazilian Amazon

Cited by 7 publications

References 22 publications

Sickle Cell Anemia Patients Display an Intricate Cellular and Serum Biomarker Network Highlighted by TCD4+CD69+ Lymphocytes, IL-17/MIP-1β, IL-12/VEGF, and IL-10/IP-10 Axis

Sickle Cell Anemia Patients Display an Intricate Cellular and Serum Biomarker Network Highlighted by TCD4+CD69+ Lymphocytes, IL-17/MIP-1β, IL-12/VEGF, and IL-10/IP-10 Axis

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018

The Anti-Sickling Properties of Medicinal Plants, Insights in Botanical Medicine*

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