Foetal Haemoglobin, Erythrocytes Containing Foetal Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell Anaemia

Author:

Tshilolo L.12,Summa V.3,Gregorj C.3,Kinsiama C.1ORCID,Bazeboso J. A.1ORCID,Avvisati G.3,Labie D.4

Affiliation:

1. Unité de Dépistage de la Drépanocytose, Centre Hospitalier Monkole, BP 817, Kinshasa XI, Democratic Republic of Congo

2. Centre de Formation et d’Appui Sanitaire (CEFA), 10, Avenue Kemi, Mont Ngafula, Kinshasa, Democratic Republic of Congo

3. Servizio di Ematologia, Università Campus Bio-Medico di Roma, 21, Via Alvaro del Portillo, 00128 Roma, Italy

4. INSERM, Institut Cochin, 4, rue du Faubourg Saint-Jacques, 75014 Paris, France

Abstract

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 (, mean age 8.9 yrs) and compared with a control group (, 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.

Publisher

Hindawi Limited

Subject

Cell Biology,Hematology

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