Human leukocyte antigen distribution and genomic ancestry in Brazilian patients with sickle cell disease

HLA ◽  
2017 ◽  
Vol 90 (4) ◽  
pp. 211-218 ◽  
Author(s):  
M. C. F. da Silva-Malta ◽  
P. S. Rodrigues ◽  
L. W. Zuccherato ◽  
F. C. B. de Souza ◽  
E. M. F. L. Domingues ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Human Leukocyte Antigen ◽  
Sickle Cell ◽  
Human Leukocyte ◽  
Cell Disease ◽  
Leukocyte Antigen

scholarly journals Non‐myeloablative human leukocyte antigen‐matched related donor transplantation in sickle cell disease: outcomes from three independent centres

2021 ◽  
Vol 192 (4) ◽  
pp. 761-768
Author(s):  
Mohsen Alzahrani ◽  
Moussab Damlaj ◽  
Neal Jeffries ◽  
Bader Alahmari ◽  
Avani Singh ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Human Leukocyte Antigen ◽  
Sickle Cell ◽  
Human Leukocyte ◽  
Cell Disease ◽  
Leukocyte Antigen ◽  
Disease Outcomes ◽  
Related Donor

scholarly journals Evidence-Based Minireview: In young children with severe sickle cell disease, do the benefits of HLA-identical sibling donor HCT outweigh the risks?

Hematology ◽  
2021 ◽  
Vol 2021 (1) ◽  
pp. 190-195
Author(s):  
Niketa Shah ◽  
Lakshmanan Krishnamurti
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Medical Condition ◽  
Human Leukocyte ◽  
Female Child ◽  
Cell Transplant ◽  
Cell Disease ◽  
Hematopoietic Stem ◽  
Febrile Episodes ◽  
First Year Of Life

Abstract In case 1, a 14-month-old male child with sickle cell disease (SCD) was referred for evaluation for an allogeneic hematopoietic stem cell transplant (HCT). The patient had a history of dactylitis 3 times in his first year of life and febrile episodes twice at the consult. His 4-year-old sister was found to be human leukocyte antigen (HLA) identical. The patient was started on hydroxyurea (HU) at 2.5 years of age. His parents again sought consultation when he was 5 years old because of concerns about his medical condition. At the time, the patient had experienced 2 vaso-occlusive pain episodes (VOEs) requiring hospitalization during the previous 2 years. He had also experienced intermittent pain crises requiring rest at home for 2 to 3 days. The child has not attended school in person due to the COVID-19 pandemic. The family is considering HCT but is ambivalent about it because of potential toxicity. In case 2, an 8-year-old female child is 3 years out from HCT for SCD from her HLA-identical sibling. Before HCT, despite receiving HU, she had experienced >5 VOEs requiring hospitalization and 2 episodes of acute chest syndromes in the previous 3 years. She had also been missing almost 50 days of school days each year. After HCT, she is now attending school regularly and participating in all normal age-appropriate activities. The parents believe that HCT has been transformative in their child's life.

scholarly journals Impact of Human Leukocyte Antigen on the Risk of Stroke in Patients with Sickle Cell Disease

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4831-4831
Author(s):  
Huda Al-Harrasi ◽  
Murtadha Al-Khabori ◽  
Salam Al-Kindi ◽  
Yasser Ahmed Mohamed Soliman Wali ◽  
Mohamed Al Huneini ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Board Of Directors ◽  
Human Leukocyte ◽  
The Other ◽  
Cell Disease ◽  
Advisory Committees ◽  
Hla Alleles ◽  
Leukocyte Antigen ◽  
Increased Risk ◽  
The Impact

Objectives: Sickle Cell Disease (SCD) is an inherited hemoglobinopathy with multiple complications including stroke. It is observed that siblings with SCD have an increased risk of stroke if there was a sibling in the family with SCD and stroke (Driscoll M C, Blood, 2003). Human Leukocyte Antigen (HLA) is extremely variable among individuals and certain HLA alleles have previously been associated with a number of diseases. It has been shown that HLA-DRB1*0301, HLA-DRB1*0302 and HLA-DQB1*0201 increase the risk of stroke and HLA-DRB1*1501 and HLA-DQB1*0602 lower the risk of stroke in patients with SCD (Styles L, Blood, 2000). These alleles are not common in all populations. We therefore planned to assess the impact of other HLA alleles on the risk of stroke in patients with SCD. Methods: This is a retrospective case-control study conducted at Sultan Qaboos University Hospital. We included all adult and pediatric patients with SCD and diagnosed with stroke using CT/MRI of the brain. The study included patients diagnosed during the period of 1995 to 2015. HLA typing was performed using molecular techniques. HLA alleles with a frequency of at least 5% were selected for analysis. Association studies were performed using Fisher Exact test. All statistical tests were performed using R program (version 3.1.2). Results: A total of 244 patients were included (66 patients with SCD and stroke [cases] and 178 patients with SCD with no stroke. The median age was 26 years (Interquartile Range [IQR]: 19-32) and 23 (IQR: 17-29 ) for cases and controls respectively. The median hemoglobin, mean corpuscular volume, hemoglobin S level and lactate dehydrogenase level was 9 g/dL, 70 fL, 70% and 400 U/L respectively for the cases, and 9 g/dL, 60 fL, 70% and 400 U/L respectively for the control. The most frequent HLA-A allele was HLA-A*02 (24%) which did not impact the risk of stroke (Odds Ratio [OR]: 1.59, 95% Confidence Interval [CI]: 0.84-3.02) as were the other tested HLA-A alleles (all CIs crossed OR of 1.00). The most frequent HLA-B allele was HLA-B*51 (15%) which did not impact the risk of stroke (OR: 0.89, 95% CI: 0.43-1.85) as were the other tested HLA-B alleles (all CIs crossed OR of 1.00). The most frequent HLA-C allele was HLA-C*04 (17%) which did not impact the risk of stroke (OR: 1.34, 95% CI: 0.67-2.65); however, HLA-C*06 (OR: 2.37, 95% CI: 1.01-5.54) increased the risk of stroke while HLA-C*07 protected from stroke (OR: 0.35, 95% CI: 0.13-0.93). None of the other HLA-C alleles were statistically significant (all CIs crossed OR of 1.00). The most frequent HLA-DRB1 allele was HLA-DRB1*16 (18%) which did not impact the risk of stroke (OR: 1.02, 95% CI:0.51-2.03) as were the other tested HLA-DRB1 alleles (all CIs crossed OR of 1.00). Conclusions: HLA-C*06 is associated with an increased risk of stroke in patients with SCD while HLA-C*07 is protective from stroke in these patients. Early identification of high-risk patients would be beneficial for preventive intervention, such as chronic transfusion or bone marrow transplantation. The impact of these HLA alleles should be validated in other populations. Disclosures Al-Khabori: Shire (Takeda): Membership on an entity's Board of Directors or advisory committees; Servier: Membership on an entity's Board of Directors or advisory committees; SOBI: Honoraria; Amgen: Membership on an entity's Board of Directors or advisory committees; AstraZeneca: Honoraria; NovoNardisk: Membership on an entity's Board of Directors or advisory committees; Roche: Membership on an entity's Board of Directors or advisory committees; Abbvie: Membership on an entity's Board of Directors or advisory committees; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees.

Abstract # OR-8: Serum 25-Hydroxyvitamin D Associates with Human Leukocyte Antigen (HLA) Polymorphisms

2016 ◽  
Vol 22 ◽  
pp. 6
Author(s):  
Leena Kinnunen ◽  
Valma Harjutsalo ◽  
Heljä-Marja Surcel ◽  
Christel Lamberg-Allardt ◽  
Jaakko Tuomilehto ◽  
...  
Keyword(s):  
Human Leukocyte Antigen ◽  
Human Leukocyte ◽  
Leukocyte Antigen ◽  
Hydroxyvitamin D ◽  
25 Hydroxyvitamin D

Hemolysis in sickle cell disease

1974 ◽  
Vol 133 (4) ◽  
pp. 624-631 ◽  
Author(s):  
T. A. Bensinger
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease
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