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Etude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients

Homozygous sickle cell disease is one of the most frequent haemoglobinopathies in Morocco. Sickle cell disease is characterized by a large clinical and biological expression variability which depends on modulating genetic and environmental factors. Clinical manifestation includes regenerative anemia...

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Autores principales: Dahmani, Fatima, Benkirane, Souad, Kouzih, Jaafar, Woumki, Aziz, Mamad, Hassan, Masrar, Azlarab
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The African Field Epidemiology Network 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5337290/
https://www.ncbi.nlm.nih.gov/pubmed/28293356
http://dx.doi.org/10.11604/pamj.2016.25.240.11118
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author Dahmani, Fatima
Benkirane, Souad
Kouzih, Jaafar
Woumki, Aziz
Mamad, Hassan
Masrar, Azlarab
author_facet Dahmani, Fatima
Benkirane, Souad
Kouzih, Jaafar
Woumki, Aziz
Mamad, Hassan
Masrar, Azlarab
author_sort Dahmani, Fatima
collection PubMed
description Homozygous sickle cell disease is one of the most frequent haemoglobinopathies in Morocco. Sickle cell disease is characterized by a large clinical and biological expression variability which depends on modulating genetic and environmental factors. Clinical manifestation includes regenerative anemia whose severity may vary among individuals. In the absence of treatment, it results in premature death. Sickle cell disease is characterized by a large clinical and biological expression variability which depends on genetic and environmental factors. A severe clinical picture marked by high early transfusion frequency, severe infectious complications and early mortality. A constant inflammatory condition characterized by elevated inflammatory proteins and compromised nutritional status. The objective of this study is to determine the hematological parameters profile in moroccan patients with homozygous sickle cell (SS) disease during stationary phases. We conducted a cross-sectional descriptive study of 87 patients with sickle cell (SS) disease. We performed a biological study based on: Hemogram with morphological assessment of red blood cells stained with MGG and automated reticulocyte counting; Hemoglobin electrophoresis test performed on alkaline agarose gel (pH 8.8) and densitometric integration. The average age is 13.22 years ± 16.36, ranging betrween 0.6 and 36 years, with a sex ratio (M/F) of 1.175. Biological effects of anemia were intense in 88.5% of patients; 67.8% of patients had normocytic anemia compared with 29.9% with microcytosis, and 2.3% with macrocytosis. The degree of anisocytosis was related to the degree of anemia, very evocative in patients with homozygous S/S (95.4%). Reticulocytosis was observed in 81.6% of patients; 52.9% of patients had thrombocytosis. Leukocytosis was observed in 64.4% of patients; 80.5% of patients had neutropenia. The parameters of the hemogram will serve as a basis for comparison during crises and will make it possible to evaluate the effectiveness of patient management. High white blood cell count, platelets and MCHC seem to be determinant of sickle cell anemia severity in Morocco. The haematological profile of moroccan patients with sickle cell disease exhibits data similar to those reported in literature relating to patients with leucocytosis from Central Africa. The results of our study suggest that sickle cell anemia is the most common health problem in Morocco and they are similar to those for major sickle cell syndrome.
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spelling pubmed-53372902017-03-14 Etude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients Dahmani, Fatima Benkirane, Souad Kouzih, Jaafar Woumki, Aziz Mamad, Hassan Masrar, Azlarab Pan Afr Med J Case Series Homozygous sickle cell disease is one of the most frequent haemoglobinopathies in Morocco. Sickle cell disease is characterized by a large clinical and biological expression variability which depends on modulating genetic and environmental factors. Clinical manifestation includes regenerative anemia whose severity may vary among individuals. In the absence of treatment, it results in premature death. Sickle cell disease is characterized by a large clinical and biological expression variability which depends on genetic and environmental factors. A severe clinical picture marked by high early transfusion frequency, severe infectious complications and early mortality. A constant inflammatory condition characterized by elevated inflammatory proteins and compromised nutritional status. The objective of this study is to determine the hematological parameters profile in moroccan patients with homozygous sickle cell (SS) disease during stationary phases. We conducted a cross-sectional descriptive study of 87 patients with sickle cell (SS) disease. We performed a biological study based on: Hemogram with morphological assessment of red blood cells stained with MGG and automated reticulocyte counting; Hemoglobin electrophoresis test performed on alkaline agarose gel (pH 8.8) and densitometric integration. The average age is 13.22 years ± 16.36, ranging betrween 0.6 and 36 years, with a sex ratio (M/F) of 1.175. Biological effects of anemia were intense in 88.5% of patients; 67.8% of patients had normocytic anemia compared with 29.9% with microcytosis, and 2.3% with macrocytosis. The degree of anisocytosis was related to the degree of anemia, very evocative in patients with homozygous S/S (95.4%). Reticulocytosis was observed in 81.6% of patients; 52.9% of patients had thrombocytosis. Leukocytosis was observed in 64.4% of patients; 80.5% of patients had neutropenia. The parameters of the hemogram will serve as a basis for comparison during crises and will make it possible to evaluate the effectiveness of patient management. High white blood cell count, platelets and MCHC seem to be determinant of sickle cell anemia severity in Morocco. The haematological profile of moroccan patients with sickle cell disease exhibits data similar to those reported in literature relating to patients with leucocytosis from Central Africa. The results of our study suggest that sickle cell anemia is the most common health problem in Morocco and they are similar to those for major sickle cell syndrome. The African Field Epidemiology Network 2016-12-20 /pmc/articles/PMC5337290/ /pubmed/28293356 http://dx.doi.org/10.11604/pamj.2016.25.240.11118 Text en © Fatima Dahmani et al. http://creativecommons.org/licenses/by/2.0/ The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Series
Dahmani, Fatima
Benkirane, Souad
Kouzih, Jaafar
Woumki, Aziz
Mamad, Hassan
Masrar, Azlarab
Etude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients
title Etude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients
title_full Etude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients
title_fullStr Etude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients
title_full_unstemmed Etude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients
title_short Etude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients
title_sort etude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients
topic Case Series
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5337290/
https://www.ncbi.nlm.nih.gov/pubmed/28293356
http://dx.doi.org/10.11604/pamj.2016.25.240.11118
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