Niwa K, Aotsuka H, Hamada H, Uchishiba M, Terai M, Niimi H
Department of Cardiology, Chiba Children's Hospital, Japan.
Coron Artery Dis. 1995 Nov;6(11):857-64.
We report on 10 patients with Kawasaki disease and thrombocytopenia who were found to have a high incidence of coronary artery aneurysm and acute myocardial infarction. The clinical features of these patients, the cause of their thrombocytopenia and the relationship between cardiovascular pathology and thrombocytopenia were analyzed.
The clinical features of 10 patients with Kawasaki disease found to have thrombocytopenia (group A: mean age 8.0 +/- 7.0 months) and those of 293 patients with Kawasaki disease (group B, controls: mean age 13 +/- 20 months) were analyzed. Coagulation studies and bone marrow aspiration were performed to ascertain the cause of the thrombocytopenia in nine out of 10 subjects in group A.
The minimum platelet count was 4-12 x 10(4)/mm3 (average day of illness, 10.3) and platelet counts were elevated to the baseline value within 1-2 weeks of onset of the illness. Low fibrinogen concentrations, high levels of fibrin degradation products, and low erythrocyte sedimentation rates with high C-reactive protein levels were observed in seven patients. In two other patients, immature megakaryocytes with normal coagulation values were observed. The differences in the incidence of coronary artery aneurysm and acute myocardial infarction between groups A and B were highly significant (coronary artery aneurysm: 60% in group A, 8.9% in group B; acute myocardial infarction: 40% in group A, 0.3% in group B).
In many patients with Kawasaki disease and thrombocytopenia, the thrombocytopenia appears to be a result of intravascular coagulation, and to be one of the risk factors for acute myocardial infarction.
我们报告了10例川崎病合并血小板减少症的患者，发现其冠状动脉瘤和急性心肌梗死的发生率较高。分析了这些患者的临床特征、血小板减少的原因以及心血管病理与血小板减少之间的关系。
分析了10例川崎病合并血小板减少症患者（A组：平均年龄8.0±7.0个月）和293例川崎病患者（B组，对照组：平均年龄13±20个月）的临床特征。对A组10名受试者中的9名进行了凝血研究和骨髓穿刺，以确定血小板减少的原因。
血小板计数最低为4 - 12×10⁴/mm³（疾病平均天数，10.3），发病后1 - 2周内血小板计数升至基线值。7例患者出现纤维蛋白原浓度降低、纤维蛋白降解产物水平升高、红细胞沉降率降低伴C反应蛋白水平升高。另外2例患者观察到未成熟巨核细胞且凝血值正常。A组和B组冠状动脉瘤和急性心肌梗死的发生率差异具有高度显著性（冠状动脉瘤：A组60%，B组8.9%；急性心肌梗死：A组40%，B组0.3%）。
在许多川崎病合并血小板减少症的患者中，血小板减少似乎是血管内凝血的结果，并且是急性心肌梗死的危险因素之一。
