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镰状细胞贫血症患儿稳定期的血液学特征

Haematological Profile of Children With Sickle Cell Anaemia in Steady State.

作者信息

Aliu Rasaki, Iliya Jalo, Quadri Oladeji R, Ibrahim Olayinka R, Daniel Ezra

机构信息

Pediatrics, Gombe State University/Federal Teaching Hospital, Gombe, NGA.

Otorhinolaryngology, Gombe State University/Federal Teaching Hospital, Gombe, NGA.

出版信息

Cureus. 2020 Oct 18;12(10):e11011. doi: 10.7759/cureus.11011.

Abstract

Introduction Sickle cell anaemia (SCA) is an inherited, autosomal recessive condition that results from a mutation in the β-globin gene. Vascular occlusion is the underlying mechanism behind a myriad of complications encountered. This vascular occlusion is primarily caused by the increased tendency of red blood cells (RBC) to adhere to the vascular endothelium, and the activation of platelets and total leucocyte count (TLC), hence the need for a steady-state haematological profile in these patients. Method This was a cross-sectional study conducted over four months at a sickle cell clinic. Haemoglobin (Hb) concentration, haematocrit, platelet, TLC, mean corpuscular volume (MCV), mean corpuscular haemoglobin (MCH) and mean corpuscular haemoglobin concentration (MCHC) of the subjects were recorded and analysed. Results Ninety-nine subjects aged 1-18 years were recruited for the study. There were 53 (53.5%) males. Leucocytosis was seen in 80 (80.8%), anaemia in 99 (100%), and thrombocytosis in 30 (30.3%) patients. The mean Hb, TLC and platelets were 7.9 ± 1.3g/dl, 14.3 ± 4.5 x 10/mm and 391.5 ± 182.6 x 10/mm respectively. Mean MCV, MCH and MCHC were 81.3 ± 7.1 fl, 28.6 ± 2.9 pg and 35.2 ± 1.7 g/dl respectively. Children aged one to four years had the highest TLC (p=0.002) but the lowest mean Hb and platelet (p=0.094 and 0.06) respectively. The mean MCV, MCH and MCHC were lowest in children aged one to four years (p=0.047, 0.001 and 0.001). Conclusion Anaemia, leucocytosis and thrombocytosis are characteristics features of children with SCA, especially in male and younger subjects. Although Iron markers are generally normal in children with SCA, those under the age of five years tend to have lower values.

摘要

引言

镰状细胞贫血(SCA)是一种遗传性常染色体隐性疾病,由β-珠蛋白基因突变引起。血管闭塞是其所引发的众多并发症的潜在机制。这种血管闭塞主要是由于红细胞(RBC)黏附于血管内皮的倾向增加,以及血小板和白细胞总数(TLC)的激活,因此这些患者需要稳定的血液学指标。

方法

这是一项在镰状细胞诊所进行了四个月的横断面研究。记录并分析了受试者的血红蛋白(Hb)浓度、血细胞比容、血小板、TLC、平均红细胞体积(MCV)、平均红细胞血红蛋白含量(MCH)和平均红细胞血红蛋白浓度(MCHC)。

结果

本研究招募了99名1至18岁的受试者。其中男性53名(53.5%)。80名(80.8%)患者出现白细胞增多,99名(100%)患者出现贫血,30名(30.3%)患者出现血小板增多。平均Hb、TLC和血小板分别为7.9±1.3g/dl、14.3±4.5×10/mm和391.5±182.6×10/mm。平均MCV、MCH和MCHC分别为81.3±7.1fl、28.6±2.9pg和35.2±1.7g/dl。1至4岁的儿童TLC最高(p = 0.002),但平均Hb和血小板最低(p = 0.094和0.06)。1至4岁儿童的平均MCV、MCH和MCHC最低(p = 0.047、0.001和0.001)。

结论

贫血、白细胞增多和血小板增多是SCA患儿的特征性表现,尤其是在男性和较年幼的受试者中。虽然SCA患儿的铁指标通常正常,但5岁以下的患儿数值往往较低。

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