Zhang Wenjing, Feng Huiqing, Zhao Jing, Li Man, Liu Yanping
Wenjing Zhang, Department of Cardiology, Baoding Hospital of Beijing Children's Hospital, Capital Medical University, 3399, Hengxiang North Street, Baoding, Hebei Province 071000, China.
Huiqing Feng, Department of Cardiology, Baoding Hospital of Beijing Children's Hospital, Capital Medical University, 3399, Hengxiang North Street, Baoding, Hebei Province 071000, China.
Pak J Med Sci. 2025 May;41(5):1299-1304. doi: 10.12669/pjms.41.5.11538.
To investigate clinical characteristics of Kawasaki disease (KD) complicated with macrophage activation syndrome (MAS) and to identify possible indicators for early diagnosis.
Clinical data, laboratory indexes, treatment, and prognosis of five patients with KD complicated with MAS admitted to Department of Cardiology, Baoding Children's Hospital from April 2020 to April 2022 were retrospectively analyzed.
Five pediatric patients aged between three months and five years were included in the study. The enrolled patients were non-reactive to intravenous immunoglobulin (IVIG) and presented with mental impairment, abdominal distension, skin rash, and scleroderma of the hands and feet. Four patients had hepatomegaly and two patients had splenomegaly. There were four patients with coronary artery diseases. Triglyceride (TG) levels were increased or showed an upward trend in four patients. Two patients had positive bone marrow hemophagocytosis. All patients had decreased fibrinogen, increased ferritin levels, a decrease or a downward trend in the natural killer (NK) lymphocytes counts, and significantly increased sCD25. Four patients presented with decreased activity of NK cells. All pediatric patients enrolled were treated with two doses of high-dose IVIG +methylprednisolone. One patient died as a result of stopping the treatment, while four remaining patients had a good prognosis.
MAS complication should be suspected in pediatric patients with KD who are non-responsive to IVIG and show signs of mental impairment, abdominal distension, scleroderma of hands and feet, or hepatosplenomegaly. We should monitor trends in blood routine, ferritin, triglyceride, fibrinogen and NK lymphocytes counts.
探讨川崎病（KD）合并巨噬细胞活化综合征（MAS）的临床特征，并确定早期诊断的可能指标。
回顾性分析2020年4月至2022年4月在保定市儿童医院心内科收治的5例KD合并MAS患儿的临床资料、实验室指标、治疗及预后情况。
研究纳入5例年龄在3个月至5岁之间的儿科患者。入选患者对静脉注射免疫球蛋白（IVIG）无反应，并出现精神障碍、腹胀、皮疹以及手足硬皮病。4例患者有肝肿大，2例患者有脾肿大。4例患者患有冠状动脉疾病。4例患者甘油三酯（TG）水平升高或呈上升趋势。2例患者骨髓噬血细胞现象呈阳性。所有患者纤维蛋白原降低、铁蛋白水平升高、自然杀伤（NK）淋巴细胞计数减少或呈下降趋势，且可溶性细胞间黏附分子-25（sCD25）显著升高。4例患者NK细胞活性降低。所有入选的儿科患者均接受了两剂大剂量IVIG+甲泼尼龙治疗。1例患者因停止治疗死亡，其余4例患者预后良好。
对IVIG无反应且出现精神障碍、腹胀、手足硬皮病或肝脾肿大迹象的KD患儿应怀疑有MAS并发症。我们应监测血常规、铁蛋白、甘油三酯、纤维蛋白原和NK淋巴细胞计数的变化趋势。
