Wei Wang1, Fangqi Gong2, Weihua Zhu1, Songling Fu1, Qing Zhang1. 1. The Children׳s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China. 2. The Children׳s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China. Electronic address: gongfangqi@zju.edu.cn.
Abstract
OBJECTIVES: To analyze the clinical characteristics, treatment, and outcomes of Kawasaki Disease (KD) patients associated with macrophage activation syndrome (MAS) and to compare two diagnostic standards (the HLH 2009 and Ravelli׳s criteria). METHODS: All of the studied patients with Kawasaki Disease (KD) were treated at The Children׳s Hospital, Zhejiang University School of Medicine, during 2007-2010. Clinical and laboratory findings were analyzed. RESULTS: In 719 KD patients, eight patients (1.11%, 81.3 ± 49.4 months, all male) were diagnosed by Ravelli׳s criteria, but only three (0.42%) patients were diagnosed by the HLH 2009 criteria. Aspartate aminotransferase increased significantly in all cases. Alanine aminotransferase, lactate dehydrogenase, and serum ferritin increased significantly in seven cases. Cytopenia and hypertriglyceridemia (>1.5mmol/L) were found in six and five cases, respectively. Hypofibrinogenemia (<1.5g/L) was found in two cases. Three cases showed evidence of hemophagocytosis, but only one case met the HLH 2009 criteria. Ectasia of the coronary arteries occurred in two cases. Seven patients were non-responsive to IVIG. One case died after the combined application of DXM, VP16, and CSA. CONCLUSIONS: MAS may be a frequently under-recognized complication of KD, because the understanding of complications and diagnostic criteria are still in progress. The HLH 2009 criteria have low sensitivity and specificity for the diagnosis of MAS complicating KD. When hepatosplenomegaly is present in KD patients with abnormal laboratory findings, such as cytopenia, liver dysfunction, hyperferritinemia, elevated serum LDH, hypofibrinogenemia, and hypertriglyceridemia, the presence of MAS should be considered.
OBJECTIVES: To analyze the clinical characteristics, treatment, and outcomes of Kawasaki Disease (KD) patients associated with macrophage activation syndrome (MAS) and to compare two diagnostic standards (the HLH 2009 and Ravelli׳s criteria). METHODS: All of the studied patients with Kawasaki Disease (KD) were treated at The Children׳s Hospital, Zhejiang University School of Medicine, during 2007-2010. Clinical and laboratory findings were analyzed. RESULTS: In 719 KDpatients, eight patients (1.11%, 81.3 ± 49.4 months, all male) were diagnosed by Ravelli׳s criteria, but only three (0.42%) patients were diagnosed by the HLH 2009 criteria. Aspartate aminotransferase increased significantly in all cases. Alanine aminotransferase, lactate dehydrogenase, and serum ferritin increased significantly in seven cases. Cytopenia and hypertriglyceridemia (>1.5mmol/L) were found in six and five cases, respectively. Hypofibrinogenemia (<1.5g/L) was found in two cases. Three cases showed evidence of hemophagocytosis, but only one case met the HLH 2009 criteria. Ectasia of the coronary arteries occurred in two cases. Seven patients were non-responsive to IVIG. One case died after the combined application of DXM, VP16, and CSA. CONCLUSIONS:MAS may be a frequently under-recognized complication of KD, because the understanding of complications and diagnostic criteria are still in progress. The HLH 2009 criteria have low sensitivity and specificity for the diagnosis of MAS complicating KD. When hepatosplenomegaly is present in KDpatients with abnormal laboratory findings, such as cytopenia, liver dysfunction, hyperferritinemia, elevated serum LDH, hypofibrinogenemia, and hypertriglyceridemia, the presence of MAS should be considered.
Authors: Leora R Feldstein; Erica B Rose; Steven M Horwitz; Jennifer P Collins; Margaret M Newhams; Mary Beth F Son; Jane W Newburger; Lawrence C Kleinman; Sabrina M Heidemann; Amarilis A Martin; Aalok R Singh; Simon Li; Keiko M Tarquinio; Preeti Jaggi; Matthew E Oster; Sheemon P Zackai; Jennifer Gillen; Adam J Ratner; Rowan F Walsh; Julie C Fitzgerald; Michael A Keenaghan; Hussam Alharash; Sule Doymaz; Katharine N Clouser; John S Giuliano; Anjali Gupta; Robert M Parker; Aline B Maddux; Vinod Havalad; Stacy Ramsingh; Hulya Bukulmez; Tamara T Bradford; Lincoln S Smith; Mark W Tenforde; Christopher L Carroll; Becky J Riggs; Shira J Gertz; Ariel Daube; Amanda Lansell; Alvaro Coronado Munoz; Charlotte V Hobbs; Kimberly L Marohn; Natasha B Halasa; Manish M Patel; Adrienne G Randolph Journal: N Engl J Med Date: 2020-06-29 Impact factor: 91.245