Kim Hyun Kwon, Kim Han Gyu, Cho Su Jin, Hong Young Mi, Sohn Sejung, Yoo Eun-Sun, Chung Wha-Soon, Ryu Kyung-Ha
Department of Pediatrics, Ewha Womans University School of Medicine, Seoul, Republic of Korea.
Pediatr Hematol Oncol. 2011 Apr;28(3):230-6. doi: 10.3109/08880018.2010.526685.
It is difficult to predict the prognosis or clinical course of secondary hemophagocytic lymphohistiocytosis (HLH) due to the various underlying causes. The authors analyzed the clinical and laboratory findings and outcomes in patients with HLH who had initially been diagnosed with Kawasaki disease (KD), and evaluated the clinical significance of each factor. Among the 21 patients with HLH, 5 had initially been diagnosed with KD and 16 had other etiologies. A comparative analysis was performed for fever duration, presence of cytopenia, serum ferritin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), triglyceride, fibrinogen, hyponatremia, reactivation, and survival rate in those HLH patients associated with KD (group I) and other causes (group II). In patients in group I, a higher level of reactivation (20%), a lower survival rate (P = .001), higher AST (P = .031) and ferritin (P = .005), and frequent hyponatremia (P = .000) were found compared to patients in group II. Interestingly, patients in group I was older than the average of age of most KD patients. A high index of suspicion on the progression from KD to HLH would be mandatory when the KD patients show elevated AST and ferritin and the presence of hyponatremia, and especially so if the patient is of older age.
由于多种潜在病因，继发性噬血细胞性淋巴组织细胞增生症（HLH）的预后或临床病程难以预测。作者分析了最初被诊断为川崎病（KD）的HLH患者的临床和实验室检查结果及预后，并评估了各因素的临床意义。在21例HLH患者中，5例最初被诊断为KD，16例有其他病因。对与KD相关的HLH患者（I组）和其他病因的HLH患者（II组）的发热持续时间、血细胞减少症、血清铁蛋白、天冬氨酸转氨酶（AST）、丙氨酸转氨酶（ALT）、甘油三酯、纤维蛋白原、低钠血症、病情复发及生存率进行了比较分析。与II组患者相比，I组患者的病情复发率更高（20%）、生存率更低（P = 0.001）、AST（P = 0.031）和铁蛋白水平更高（P = 0.005），且低钠血症更为常见（P = 0.000）。有趣的是，I组患者的年龄高于大多数KD患者的平均年龄。当KD患者出现AST和铁蛋白升高以及低钠血症时，尤其是患者年龄较大时，必须高度怀疑其病情已从KD进展为HLH。
