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...

Full description

Bibliographic Details
Main Authors: L. Tshilolo, V. Summa, C. Gregorj, C. Kinsiama, J. A. Bazeboso, G. Avvisati, D. Labie
Format: Article
Language:English
Published: Hindawi Limited 2012-01-01
Series:Anemia
Online Access:http://dx.doi.org/10.1155/2012/105349
id doaj-2944064457d84cf381e002a27b8cb817
record_format Article
spelling 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
work_keys_str_mv AT ltshilolo foetalhaemoglobinerythrocytescontainingfoetalhaemoglobinandhematologicalfeaturesincongolesepatientswithsicklecellanaemia
AT vsumma foetalhaemoglobinerythrocytescontainingfoetalhaemoglobinandhematologicalfeaturesincongolesepatientswithsicklecellanaemia
AT cgregorj foetalhaemoglobinerythrocytescontainingfoetalhaemoglobinandhematologicalfeaturesincongolesepatientswithsicklecellanaemia
AT ckinsiama foetalhaemoglobinerythrocytescontainingfoetalhaemoglobinandhematologicalfeaturesincongolesepatientswithsicklecellanaemia
AT jabazeboso foetalhaemoglobinerythrocytescontainingfoetalhaemoglobinandhematologicalfeaturesincongolesepatientswithsicklecellanaemia
AT gavvisati foetalhaemoglobinerythrocytescontainingfoetalhaemoglobinandhematologicalfeaturesincongolesepatientswithsicklecellanaemia
AT dlabie foetalhaemoglobinerythrocytescontainingfoetalhaemoglobinandhematologicalfeaturesincongolesepatientswithsicklecellanaemia
_version_ 1725445077587722240