Macrophage activation syndrome in a newborn of adult-onset Still's disease mother: a case report and literature review
10.3760/cma.j.issn.2096-2932.2018.06.011
- VernacularTitle:母亲成人Still病伴发新生儿巨噬细胞活化综合征一例报告并文献复习
- Author:
Fei BEI
1
;
Yanliang JIN
;
Jianhua SUN
;
Huiqun JU
;
Hua HUANG
Author Information
1. 200127,上海交通大学医学院附属上海儿童医学中心新生儿科
- Keywords:
Macrophage activation syndrome;
Lymphohistiocytosis,hemophagocytic;
Autoimmune diseases;
Maternal exposure
- From:Chinese Journal of Neonatology
2018;33(6):446-449
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the clinical characteristics of neonatal macrophage activation syndrome (MAS) associated with maternal rheumatic diseases and improve the understanding of neonatal MAS.Method Clinical data of MAS in a newborn infant with adult-onset Still's disease (AOSD) mother was retrospectively studied.From the establishment day of databases (CNKI,VIP,Wanfang,Pubmed and Embase) to December 2017,literature were retrieved with key words including "newborn","macrophage activation syndrome" and "hemophagocytic lymphohistiocytosis (HLH) ".Clinical features of infant MAS/HLH with maternal rheumatic diseases were summarized.Result A 27-day-old boy with AOSD mother manifested with fever,watery stools,irritability,prominent enlargement of right parotid gland and right cervical lymphadenitis.The infant was diagnosed with MAS due to coagulopathy,multiple organ dysfunction,hypofibrinogenemia and increased levels of ferritin.Anti-SSA/Ro52kD and stool rotavims antigen were positive.The infant recovered with intravenous immunoglobulin and steroids therapy.Follow-up at 2-year-old were normal.A total of 3 other cases of neonatal MAS/HLH were retrieved.All patients had high fever,hepatosplenomegaly and multiple organ dysfunction,impaired digestive system (abdominal distention,diarrhea and ascites),disseminated intravascular coagulation (2 cases),mental disorders (1 case),complete atrioventricular block (1 case) and severe hypotension (1 case).Laboratory results showed thrombocytopenia,elevated level of hepatic enzyme and serum ferritin in all patients.Targeted panel-based next generation sequencing were all negative for pathogenic gene mutations.After treatments of steroids,intravenous immunoglobulin and chemotherapy,all patients improved and ultimately cured.Conclusion In view of the impacts of the maternal rheumatic diseases on fetus,newborns with early onset high fever and hepatosplenomegaly should be suspected of MAS.Early diagnosis and effective treatment are crucial for clinical improvement.
