Matter Randa M, Ragab Iman A, Roushdy Alaa M, Ahmed Ahmed G, Aly Hanan H, Ismail Eman A
Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Cardiology Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Congenit Heart Dis. 2018 Jan;13(1):118-123. doi: 10.1111/chd.12530. Epub 2017 Sep 7.
Congenital heart defects are common noninfectious causes of mortality in children. Bleeding and thrombosis are both limiting factors in the management of such patients. We assessed the frequency of thrombocytopenia in pediatric patients with congenital cyanotic heart disease (CCHD) and evaluated determinants of platelet count including immature platelet fraction (IPF) and their role in the pathogenesis of thrombocytopenia.
Forty-six children and adolescents with CCHD during pre-catheter visits were studied; median age was 20.5 months. Complete blood count including IPF as a marker of platelet production and reticulated hemoglobin content (RET-He) as a marker of red cell production and iron status were done on Sysmex XE 2100 (Sysmex, Japan). C-reactive protein, prothrombin time (PT), Activated partial thromboplastin time (APTT) were also assessed.
Thrombocytopenia was found in 6 patients (13%). PT was prolonged (P = .016) and IPF was significantly higher in patients with thrombocytopenia compared with patients with normal platelet count (14.15 ± 5.2% vs 6.68 ± 3.39%; P = .003). Platelet count was negatively correlated with IPF while significant positive correlations were found between IPF and hemoglobin, red blood cells (RBCs) count, hematocrit (Hct), PT, reticulocytes count, and immature reticulocyte fraction.
We suggest that elevated IPF in CCHD patients with thrombocytopenia may denote peripheral platelets destruction as an underlying mechanism. Hemoglobin level, RBCs count, Hct, and RET-He were not significant determinants for platelet count in CCHD.
先天性心脏病是儿童常见的非感染性死亡原因。出血和血栓形成都是此类患者治疗中的限制因素。我们评估了先天性紫绀型心脏病(CCHD)患儿血小板减少症的发生率,并评估了包括未成熟血小板分数(IPF)在内的血小板计数的决定因素及其在血小板减少症发病机制中的作用。
对46例在导管检查前就诊的CCHD儿童和青少年进行研究;中位年龄为20.5个月。使用Sysmex XE 2100(日本Sysmex公司)进行全血细胞计数,包括作为血小板生成标志物的IPF以及作为红细胞生成和铁状态标志物的网织血红蛋白含量(RET-He)。还评估了C反应蛋白、凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)。
6例患者(13%)出现血小板减少症。与血小板计数正常的患者相比,血小板减少症患者的PT延长(P = 0.016),IPF显著更高(14.15±5.2% vs 6.68±3.39%;P = 0.003)。血小板计数与IPF呈负相关,而IPF与血红蛋白、红细胞(RBC)计数、血细胞比容(Hct)、PT、网织红细胞计数和未成熟网织红细胞分数之间存在显著正相关。
我们认为,血小板减少的CCHD患者IPF升高可能表明外周血小板破坏是潜在机制。血红蛋白水平、RBC计数、Hct和RET-He不是CCHD患者血小板计数的重要决定因素。