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Human platelet alloantigens (HPA) 1, HPA2, HPA3, HPA4, and HPA5 polymorphisms in sickle cell anemia patients with vaso-occlusive crisis.
Eur J Haematol. 2009 Dec 01; 83(6):579-85.EJ

Abstract

OBJECTIVES

Vaso-occlusive crisis (VOC) is a significant cause of morbidity and mortality in sickle cell anemia (SCA) patients. Insofar as polymorphism in human platelet alloantigen (HPA) exhibit a prothrombotic nature, we hypothesized that specific HPA polymorphic variants are associated with VOC. We investigated the distribution of HPA1, HPA2, HPA3, HPA4, and HPA5 alleles genotypes among VOC and non-VOC control SCA patients.

PATIENTS/METHODS

This was a case-control study. Study subjects comprised SCA patients with (VOC group; n = 127) or without (Steady-state group; n = 130) VOC events. HPA genotyping was done by PCR-SSP.

RESULTS

Significantly higher frequencies of HPA-2b, HPA-3b, and HPA-5b alleles, and marked enrichment of HPA-3b/3b, HPA-5a/5b, and HPA-5b/5b genotypes, were seen in VOC than in control SCA patients. Taking homozygous wild-type genotypes as reference, univariate analysis identified HPA-3a/3b, HPA-3b/3b, and HPA-5b/5b to be associated with VOC. Multivariate analysis confirmed the independent association of only HPA-3a/3b and HPA-3b/3b genotypes with VOC. HPA-3 genotypes were significantly correlated with VOC frequency, type, and medication, and requirement for hospitalization. While both HPA 3a/3b (P = 0.002; OR = 2.94; 95% CI = 1.49-5.77) and 3b/3b (P = 0.006; OR = 3.16; 95% CI = 1.40-7.17) genotypes were associated with need for hospitalization, only HPA-3b/3b was associated with VOC frequency, type (localized vs. generalized), and medication (narcotics vs. NSAIDs).

CONCLUSION

This confirms the association of HPA polymorphisms with SCA VOC, of which HPA-3 appears to be independent genetic risk factors for SCA VOC.

Authors+Show Affiliations

Department of Medical Biochemistry, Arabian Gulf University, Manama, Bahrain.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

19702628

Citation

Al-Subaie, Abeer M., et al. "Human Platelet Alloantigens (HPA) 1, HPA2, HPA3, HPA4, and HPA5 Polymorphisms in Sickle Cell Anemia Patients With Vaso-occlusive Crisis." European Journal of Haematology, vol. 83, no. 6, 2009, pp. 579-85.
Al-Subaie AM, Fawaz NA, Mahdi N, et al. Human platelet alloantigens (HPA) 1, HPA2, HPA3, HPA4, and HPA5 polymorphisms in sickle cell anemia patients with vaso-occlusive crisis. Eur J Haematol. 2009;83(6):579-85.
Al-Subaie, A. M., Fawaz, N. A., Mahdi, N., Al-Absi, I. K., Al-Ola, K., Ameen, G., & Almawi, W. Y. (2009). Human platelet alloantigens (HPA) 1, HPA2, HPA3, HPA4, and HPA5 polymorphisms in sickle cell anemia patients with vaso-occlusive crisis. European Journal of Haematology, 83(6), 579-85. https://doi.org/10.1111/j.1600-0609.2009.01339.x
Al-Subaie AM, et al. Human Platelet Alloantigens (HPA) 1, HPA2, HPA3, HPA4, and HPA5 Polymorphisms in Sickle Cell Anemia Patients With Vaso-occlusive Crisis. Eur J Haematol. 2009 Dec 1;83(6):579-85. PubMed PMID: 19702628.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Human platelet alloantigens (HPA) 1, HPA2, HPA3, HPA4, and HPA5 polymorphisms in sickle cell anemia patients with vaso-occlusive crisis. AU - Al-Subaie,Abeer M, AU - Fawaz,Naglaa A, AU - Mahdi,Najat, AU - Al-Absi,Iman K, AU - Al-Ola,Khadija, AU - Ameen,Ghada, AU - Almawi,Wassim Y, Y1 - 2009/08/21/ PY - 2009/8/26/entrez PY - 2009/8/26/pubmed PY - 2010/3/13/medline SP - 579 EP - 85 JF - European journal of haematology JO - Eur. J. Haematol. VL - 83 IS - 6 N2 - OBJECTIVES: Vaso-occlusive crisis (VOC) is a significant cause of morbidity and mortality in sickle cell anemia (SCA) patients. Insofar as polymorphism in human platelet alloantigen (HPA) exhibit a prothrombotic nature, we hypothesized that specific HPA polymorphic variants are associated with VOC. We investigated the distribution of HPA1, HPA2, HPA3, HPA4, and HPA5 alleles genotypes among VOC and non-VOC control SCA patients. PATIENTS/METHODS: This was a case-control study. Study subjects comprised SCA patients with (VOC group; n = 127) or without (Steady-state group; n = 130) VOC events. HPA genotyping was done by PCR-SSP. RESULTS: Significantly higher frequencies of HPA-2b, HPA-3b, and HPA-5b alleles, and marked enrichment of HPA-3b/3b, HPA-5a/5b, and HPA-5b/5b genotypes, were seen in VOC than in control SCA patients. Taking homozygous wild-type genotypes as reference, univariate analysis identified HPA-3a/3b, HPA-3b/3b, and HPA-5b/5b to be associated with VOC. Multivariate analysis confirmed the independent association of only HPA-3a/3b and HPA-3b/3b genotypes with VOC. HPA-3 genotypes were significantly correlated with VOC frequency, type, and medication, and requirement for hospitalization. While both HPA 3a/3b (P = 0.002; OR = 2.94; 95% CI = 1.49-5.77) and 3b/3b (P = 0.006; OR = 3.16; 95% CI = 1.40-7.17) genotypes were associated with need for hospitalization, only HPA-3b/3b was associated with VOC frequency, type (localized vs. generalized), and medication (narcotics vs. NSAIDs). CONCLUSION: This confirms the association of HPA polymorphisms with SCA VOC, of which HPA-3 appears to be independent genetic risk factors for SCA VOC. SN - 1600-0609 UR - https://www.unboundmedicine.com/medline/citation/19702628/Human_platelet_alloantigens__HPA__1_HPA2_HPA3_HPA4_and_HPA5_polymorphisms_in_sickle_cell_anemia_patients_with_vaso_occlusive_crisis_ L2 - https://doi.org/10.1111/j.1600-0609.2009.01339.x DB - PRIME DP - Unbound Medicine ER -