García-Pavón Susana, Yamazaki-Nakashimada Marco A, Báez Milton, Borjas-Aguilar Karla L, Murata Chiharu
*Allergy and Immunology Department, Naval Hospital of High Speciality †Clinical Immunology Department, National Institute of Pediatrics ‡Pediatric Private Practice §Research Methodology Department, National Institute of Pediatrics.
J Pediatr Hematol Oncol. 2017 Aug;39(6):445-451. doi: 10.1097/MPH.0000000000000872.
Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is a rare and potentially fatal complication of Kawasaki disease (KD). We report 2 cases, performed a literature search, and analyze the characteristics of MAS associated with KD. A total of 69 patients were evaluated, 34 reported the date of the diagnosis of MAS and KD, 6% had a diagnosis of MAS before KD, 21% had a simultaneous presentation, and 73% had the diagnosis of MAS after KD. Different treatment approaches were observed with corticosteroids administered in 87%, cyclosporine in 49%, etoposide (VP-16) in 39%, and monoclonal anti-TNF in 6% of cases. Coronary abnormalities were especially high in this group of patients (46%) and 9 patients died (13%). The persistence of fever with splenomegaly, hyperferritinemia, thrombocytopenia, and elevated aspartate aminotransferase (AST) should prompt the consideration of MAS complicating KD.
巨噬细胞活化综合征（MAS），也称为继发性噬血细胞性淋巴组织细胞增生症，是川崎病（KD）一种罕见且可能致命的并发症。我们报告2例病例，进行了文献检索，并分析了与KD相关的MAS的特征。总共评估了69例患者，34例报告了MAS和KD的诊断日期，6%在KD之前诊断为MAS，21%同时出现，73%在KD之后诊断为MAS。观察到不同的治疗方法，87%的病例使用了皮质类固醇，49%使用了环孢素，39%使用了依托泊苷（VP - 16），6%使用了单克隆抗TNF。该组患者冠状动脉异常尤其高（46%），9例患者死亡（13%）。持续发热伴脾肿大、高铁蛋白血症、血小板减少和天冬氨酸转氨酶（AST）升高应促使考虑MAS并发KD。
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