The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival.
BACKGROUND: Co-inheritance of α-thalassemia was reported to be associated with a delayed age of disease onset among Cameroonian Sickle Cell Anemia (SCA) patients. The present study aimed to explore the correlation between α-thalassemia, hematological indices, and clinical events in these patients. M...
Main Authors: | , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Public Library of Science (PLoS)
2014-01-01
|
Series: | PLoS ONE |
Online Access: | http://europepmc.org/articles/PMC4076272?pdf=render |
id |
doaj-f9cde1a8dfa549eabf8ec780e3b5636a |
---|---|
record_format |
Article |
spelling |
doaj-f9cde1a8dfa549eabf8ec780e3b5636a2020-11-25T01:20:38ZengPublic Library of Science (PLoS)PLoS ONE1932-62032014-01-0196e10051610.1371/journal.pone.0100516The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival.Maryam Bibi RumaneyValentina Josiane Ngo BitounguiAnna Alvera VorsterRaj RamesarAndre Pascal KengneJeanne NgogangAmbroise WonkamBACKGROUND: Co-inheritance of α-thalassemia was reported to be associated with a delayed age of disease onset among Cameroonian Sickle Cell Anemia (SCA) patients. The present study aimed to explore the correlation between α-thalassemia, hematological indices, and clinical events in these patients. METHODS AND FINDINGS: We studied 161 Cameroonian SCA patients and 103 controls (59.1% HbAA) with median ages of 17.5 and 23 years. RFLP-PCR was used to confirm SCA genotype and to describe haplotypes in the HBB-like genes cluster. Multiplex Gap-PCR was performed to investigate the 3.7 kb α-globin gene deletions. SNaPshot PCR, capillary electrophoresis and cycle sequencing were used for the genotyping of 10 SNPs in BCL11A, HMIP1/2, OR51B5/6 and HBG loci, known to influence HbF levels. Generalised linear regression models adjusted for age, sex and SNPs genotypes was used to investigate effects of α-thalassemia on clinical and hematological indices. The median rate of vaso-occlusive painful crisis and hospitalisations was two and one per year, respectively. Stroke was reported in eight cases (7.4%). Benin haplotype was the most prevalent (66.3%; n = 208 chromosomes). Among patients, 37.3% (n = 60) had at least one 3.7 kb deletion, compared to 10.9% (n = 6) among HbAA controls (p<0.001). Among patients, the median RBC count increased with the number of 3.7 kb deletions [2.6, 3.0 and 3.4 million/dl, with no, one and two deletions (p = 0.01)]. The median MCV decreased with the number of 3.7 kb deletion [86, 80, and 68fl, with no, one and two deletions (p<0.0001)], as well as median WBC counts [13.2, 10.5 and 9.8×109/L (p<0.0001. The co-inheritance of α-thalassemia was associated with lower consultations rate (p = 0.038). CONCLUSION: The co-inheritance of α-thalassemia and SCA is associated with improved hematological indices, and lower consultations rate in this group of patients. This could possibly improve their survival and explain the higher proportion of α-thalassemia among patients than controls.http://europepmc.org/articles/PMC4076272?pdf=render |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Maryam Bibi Rumaney Valentina Josiane Ngo Bitoungui Anna Alvera Vorster Raj Ramesar Andre Pascal Kengne Jeanne Ngogang Ambroise Wonkam |
spellingShingle |
Maryam Bibi Rumaney Valentina Josiane Ngo Bitoungui Anna Alvera Vorster Raj Ramesar Andre Pascal Kengne Jeanne Ngogang Ambroise Wonkam The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival. PLoS ONE |
author_facet |
Maryam Bibi Rumaney Valentina Josiane Ngo Bitoungui Anna Alvera Vorster Raj Ramesar Andre Pascal Kengne Jeanne Ngogang Ambroise Wonkam |
author_sort |
Maryam Bibi Rumaney |
title |
The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival. |
title_short |
The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival. |
title_full |
The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival. |
title_fullStr |
The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival. |
title_full_unstemmed |
The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival. |
title_sort |
co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in cameroonian patients and could improve their survival. |
publisher |
Public Library of Science (PLoS) |
series |
PLoS ONE |
issn |
1932-6203 |
publishDate |
2014-01-01 |
description |
BACKGROUND: Co-inheritance of α-thalassemia was reported to be associated with a delayed age of disease onset among Cameroonian Sickle Cell Anemia (SCA) patients. The present study aimed to explore the correlation between α-thalassemia, hematological indices, and clinical events in these patients. METHODS AND FINDINGS: We studied 161 Cameroonian SCA patients and 103 controls (59.1% HbAA) with median ages of 17.5 and 23 years. RFLP-PCR was used to confirm SCA genotype and to describe haplotypes in the HBB-like genes cluster. Multiplex Gap-PCR was performed to investigate the 3.7 kb α-globin gene deletions. SNaPshot PCR, capillary electrophoresis and cycle sequencing were used for the genotyping of 10 SNPs in BCL11A, HMIP1/2, OR51B5/6 and HBG loci, known to influence HbF levels. Generalised linear regression models adjusted for age, sex and SNPs genotypes was used to investigate effects of α-thalassemia on clinical and hematological indices. The median rate of vaso-occlusive painful crisis and hospitalisations was two and one per year, respectively. Stroke was reported in eight cases (7.4%). Benin haplotype was the most prevalent (66.3%; n = 208 chromosomes). Among patients, 37.3% (n = 60) had at least one 3.7 kb deletion, compared to 10.9% (n = 6) among HbAA controls (p<0.001). Among patients, the median RBC count increased with the number of 3.7 kb deletions [2.6, 3.0 and 3.4 million/dl, with no, one and two deletions (p = 0.01)]. The median MCV decreased with the number of 3.7 kb deletion [86, 80, and 68fl, with no, one and two deletions (p<0.0001)], as well as median WBC counts [13.2, 10.5 and 9.8×109/L (p<0.0001. The co-inheritance of α-thalassemia was associated with lower consultations rate (p = 0.038). CONCLUSION: The co-inheritance of α-thalassemia and SCA is associated with improved hematological indices, and lower consultations rate in this group of patients. This could possibly improve their survival and explain the higher proportion of α-thalassemia among patients than controls. |
url |
http://europepmc.org/articles/PMC4076272?pdf=render |
work_keys_str_mv |
AT maryambibirumaney thecoinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT valentinajosianengobitoungui thecoinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT annaalveravorster thecoinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT rajramesar thecoinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT andrepascalkengne thecoinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT jeannengogang thecoinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT ambroisewonkam thecoinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT maryambibirumaney coinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT valentinajosianengobitoungui coinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT annaalveravorster coinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT rajramesar coinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT andrepascalkengne coinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT jeannengogang coinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival AT ambroisewonkam coinheritanceofalphathalassemiaandsicklecellanemiaisassociatedwithbetterhematologicalindicesandlowerconsultationsrateincameroonianpatientsandcouldimprovetheirsurvival |
_version_ |
1725132886469771264 |