Foetal Haemoglobin, Erythrocytes Containing Foetal Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell Anaemia
High HbF levels and F cells are correlated with reduced morbidity and mortality in sickle cell disease (SCD). This paper was designed to determine the HbF and F cells levels in Congolese sickle cell anemia (SCA) patients in order to determine their impact on the expression of SCD. Population and Met...
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doaj-2944064457d84cf381e002a27b8cb8172020-11-25T00:00:26ZengHindawi LimitedAnemia2090-12672090-12752012-01-01201210.1155/2012/105349105349Foetal Haemoglobin, Erythrocytes Containing Foetal Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell AnaemiaL. Tshilolo0V. Summa1C. Gregorj2C. Kinsiama3J. A. Bazeboso4G. Avvisati5D. Labie6Unité de Dépistage de la Drépanocytose, Centre Hospitalier Monkole, BP 817, Kinshasa XI, Democratic Republic of CongoServizio di Ematologia, Università Campus Bio-Medico di Roma, 21, Via Alvaro del Portillo, 00128 Roma, ItalyServizio di Ematologia, Università Campus Bio-Medico di Roma, 21, Via Alvaro del Portillo, 00128 Roma, ItalyUnité de Dépistage de la Drépanocytose, Centre Hospitalier Monkole, BP 817, Kinshasa XI, Democratic Republic of CongoUnité de Dépistage de la Drépanocytose, Centre Hospitalier Monkole, BP 817, Kinshasa XI, Democratic Republic of CongoServizio di Ematologia, Università Campus Bio-Medico di Roma, 21, Via Alvaro del Portillo, 00128 Roma, ItalyINSERM, Institut Cochin, 4, rue du Faubourg Saint-Jacques, 75014 Paris, FranceHigh HbF levels and F cells are correlated with reduced morbidity and mortality in sickle cell disease (SCD). This paper was designed to determine the HbF and F cells levels in Congolese sickle cell anemia (SCA) patients in order to determine their impact on the expression of SCD. Population and Method. HbF levels were measured in 89 SCA patients (mean age 11.4 yrs) using a standard HPLC method. F cell quantitation was done in a second group of SCA patients (𝑛=42, mean age 8.9 yrs) and compared with a control group (𝑛=47, mean age 5 yrs). F cells were quantified by a cytofluorometric system (MoAb-HbF—FITC; cut off at 0.5%). Results. The mean value of HbF was 7.2% ± 5.0 with heterogeneous distribution, most patients (76%) having HbF < 8%. Mean values of F-cells in SCA patients and control group were 5.4% ± 7.6 (median: 2.19% ; range 0,0–30,3%) and 0.5% ± 1.6 (median 0.0, range 0–5.18), respectively. SCA patients with F cells >4.5% developed less painful crisis and had higher percentage of reticulocytes. Conclusion. Congolese SCA patients displayed low levels of HbF and F-cells that contribute to the severity of SCD.http://dx.doi.org/10.1155/2012/105349 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
L. Tshilolo V. Summa C. Gregorj C. Kinsiama J. A. Bazeboso G. Avvisati D. Labie |
spellingShingle |
L. Tshilolo V. Summa C. Gregorj C. Kinsiama J. A. Bazeboso G. Avvisati D. Labie Foetal Haemoglobin, Erythrocytes Containing Foetal Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell Anaemia Anemia |
author_facet |
L. Tshilolo V. Summa C. Gregorj C. Kinsiama J. A. Bazeboso G. Avvisati D. Labie |
author_sort |
L. Tshilolo |
title |
Foetal Haemoglobin, Erythrocytes Containing Foetal Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell Anaemia |
title_short |
Foetal Haemoglobin, Erythrocytes Containing Foetal Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell Anaemia |
title_full |
Foetal Haemoglobin, Erythrocytes Containing Foetal Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell Anaemia |
title_fullStr |
Foetal Haemoglobin, Erythrocytes Containing Foetal Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell Anaemia |
title_full_unstemmed |
Foetal Haemoglobin, Erythrocytes Containing Foetal Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell Anaemia |
title_sort |
foetal haemoglobin, erythrocytes containing foetal haemoglobin, and hematological features in congolese patients with sickle cell anaemia |
publisher |
Hindawi Limited |
series |
Anemia |
issn |
2090-1267 2090-1275 |
publishDate |
2012-01-01 |
description |
High HbF levels and F cells are correlated with reduced morbidity and mortality in sickle cell disease (SCD). This paper was designed to determine the HbF and F cells levels in Congolese sickle cell anemia (SCA) patients in order to determine their impact on the expression of SCD. Population and Method. HbF levels were measured in 89 SCA patients (mean age 11.4 yrs) using a standard HPLC method. F cell quantitation was done in a second group of SCA patients (𝑛=42, mean age 8.9 yrs) and compared with a control group (𝑛=47,
mean age 5 yrs). F cells were quantified by a cytofluorometric system (MoAb-HbF—FITC; cut off at 0.5%). Results. The mean value of HbF was 7.2% ± 5.0 with heterogeneous distribution, most patients (76%) having HbF < 8%. Mean values of F-cells in SCA patients and control group were 5.4% ± 7.6 (median: 2.19% ; range 0,0–30,3%) and 0.5% ± 1.6 (median 0.0, range 0–5.18), respectively. SCA patients with F cells >4.5% developed less painful crisis and had higher percentage of reticulocytes. Conclusion. Congolese SCA patients displayed low levels of HbF and F-cells that contribute to the severity of SCD. |
url |
http://dx.doi.org/10.1155/2012/105349 |
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