Dahmani Fatima, Benkirane Souad, Kouzih Jaafar, Woumki Aziz, Mamad Hassan, Masrar Azlarab
Équipe de Recherche en Hématologie, Laboratoire d'Hématologie, Faculté de Médecine et de Pharmacie, Université Mohammed V, Rabat, Maroc; Laboratoire Central d'Hématologie, Centre Hospitalier Ibn Sina, Rabat, Maroc.
Laboratoire Central d'Hématologie, Centre Hospitalier Ibn Sina, Rabat, Maroc.
Pan Afr Med J. 2016 Dec 20;25:240. doi: 10.11604/pamj.2016.25.240.11118. eCollection 2016.
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.
纯合子镰状细胞病是摩洛哥最常见的血红蛋白病之一。镰状细胞病的特点是临床和生物学表现具有很大的变异性,这取决于调节性遗传和环境因素。临床表现包括再生障碍性贫血,其严重程度在个体间可能有所不同。在没有治疗的情况下,会导致过早死亡。镰状细胞病的特点是临床和生物学表现具有很大的变异性,这取决于遗传和环境因素。严重的临床症状表现为早期输血频率高、严重感染并发症和早期死亡率。一种持续的炎症状态,其特征为炎症蛋白升高和营养状况受损。本研究的目的是确定摩洛哥纯合子镰状细胞(SS)病患者稳定期的血液学参数概况。我们对87例镰状细胞(SS)病患者进行了一项横断面描述性研究。我们基于以下内容进行了生物学研究:用MGG染色对红细胞进行形态学评估并自动计数网织红细胞的血常规;在碱性琼脂糖凝胶(pH 8.8)上进行血红蛋白电泳试验并进行光密度积分。平均年龄为13.22岁±16.36岁,年龄范围在0.6岁至36岁之间,性别比(男/女)为1.175。88.5%的患者贫血的生物学效应强烈;67.8%的患者为正细胞性贫血,29.9%为小细胞性贫血,2.3%为大细胞性贫血。红细胞大小不均一程度与贫血程度相关,在纯合子S/S患者中非常明显(95.4%)。81.6%的患者观察到网织红细胞增多;52.9%的患者有血小板增多。64.4%的患者观察到白细胞增多;80.5%的患者有中性粒细胞减少。血常规参数将作为危机期间比较的基础,并有助于评估患者管理的有效性。高白细胞计数、血小板和平均血红蛋白浓度似乎是摩洛哥镰状细胞贫血严重程度的决定因素。摩洛哥镰状细胞病患者的血液学特征与文献中报道的中非白细胞增多患者的数据相似。我们的研究结果表明,镰状细胞贫血是摩洛哥最常见的健康问题,且与主要镰状细胞综合征的结果相似。