Clinical features of the children with malignancy-associated hemophagocytic syndrome in Beijing
10.3760/cma.j.issn.2095-428X.2019.23.013
- VernacularTitle: 北京地区儿童肿瘤相关噬血细胞综合征临床特征分析
- Author:
Zhizhuo HUANG
1
;
Zhao WANG
2
;
Rui ZHANG
3
;
Xiaodong SHI
4
;
Ying LIU
5
;
Wanling SUN
6
;
Leping ZHANG
1
Author Information
1. Department of Pediatrics, Peking University People′s Hospital, Beijing 100044, China
2. Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China
3. Hematology Center, Beijing Children′s Hospital, Capital Medical University, Beijing 100045, China
4. Department of Hematology, the Affiliated Children′s Hospital, Capital Institute of Pediatrics, Beijing 100020, China
5. Department of Pediatrics, Chinese People′s Liberation Army General Hospital, Beijing 100853, China
6. Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China
- Publication Type:Journal Article
- Keywords:
Malignancy-associated hemophagocytic syndrome;
Child;
Clinical features;
Treatment
- From:
Chinese Journal of Applied Clinical Pediatrics
2019;34(23):1812-1815
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical features, treatment and prognosis of the children with malignancy-associated hemophagocytic syndrome (MAHS) in Beijing in recent decade.
Methods:The clinical data of the patients with MAHS under 18 years old from July 2007 to February 2018 collected by the Society of Beijing hemophagocytic syndrome were analyzed retrospectively.
Results:There were 46 patients under 18 years old with MAHS in all(male 27, female 19). The patients with MAHS took up 8.9% of the patients with hemophagocytic lymphohistiocytosis (HLH)(46/519 cases) from the area during that period.The median age of onset had 13.5 years (0.9-18.0 years). Thirty-five patients had lymphoma (76.0%), 9 cases had leukemia (19.6%), 1 case had myelodysplastic syndrome with refractory anemia with excess blast(RAEB-T), and 1 case had Epstein-Barr virus(EBV) associated lymphoproliferative disease (borderline tumor stage). All the patients had a fever.A half of them suffered from hepatosplenomegaly and 7 patients(15.2%) had neurological symptoms.The common laboratory abnormalities included cytopenias, hemophagocytosis in bone marrow (81.8%, 36/44 cases), elevated serum ferritin (87.8%, 36/41 cases), and elevated sCD25 (100.0%, 15/15 cases), decreased nature killer(NK) activity (61.1%, 11/18 cases), and plasma EBV-DNA positive (57.9%). Four patients did not receive treatment, the rest were treated by several chemotherapy protocols including the HLH-94/2004 protocol.Five patients (10.8%) received the allogeneic hematopoietic stem cell transplantation, and 1 case received the splenectomy therapy.The mortality was 58.7%.Four heterozygous mutations of SH2D1A, UNC13D and PRF1 genes were found in 2 patients.
Conclusions:MAHS in children progresses rapidly, with poor prognosis, and it is often complicated with EBV infection.The children with MAHS may develop genetic defects similar to primary hemophagocytic syndrome.Nowadays, there is no standard treatment for MAHS, so the individualized treatment is to be explored.
