Clinical Research and Practice
Analysis of 9 cases of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis
Zhao Yunze, Ma Honghao, Wang Dong, Lian Hongyun, Wang Tianyou, Zhang Rui
Published 2024-01-02
Cite as Chin J Pediatr, 2024, 62(1): 60-65. DOI: 10.3760/cma.j.cn112140-20231023-00317
Abstract
ObjectiveTo analyze the clinical features,treatment and prognosis of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis (DIHS-HLH).
MethodsThis was a retrospective case study. Clinical characteristics, laboratory results, treatment and prognosis of 9 patients diagnosed with DIHS-HLH in Beijing Children′s hospital between January 2020 and December 2022 were summarized. Kaplan-Meier survival analysis was used to calculate the overall survival rate.
ResultsAmong all 9 cases, there were 6 males and 3 females, with the age ranged from 0.8 to 3.1 years. All patients had fever, rash, hepatomegaly and multiple lymph node enlargement. Other manifestations included splenomegaly (4 cases), pulmonary imaging abnormalities (6 cases), central nervous system symptoms (3 cases), and watery diarrhea (3 cases). Most patients showed high levels of soluble-CD25 (8 cases), hepatic dysfunction (7 cases) and hyperferritinemia (7 cases). Other laboratory abnormalities included hemophagocytosis in bone marrow (5 cases), hypofibrinogenemia (3 cases) and hypertriglyceridemia (2 cases). Ascending levels of interleukin (IL) 5, IL-8 and interferon-γ (IFN-γ) were detected in more than 6 patients. All patients received high dose intravenous immunoglobulin, corticosteroid and ruxolitinib, among which 4 patients were also treated with high dose methylprednisolone, 2 patients with etoposide and 2 patients with cyclosporin A. After following up for 0.2-38.6 months, 7 patients survived, and the 1-year overall survival rate was (78±14)%. Two patients who had no response to high dose immunoglobulin, methylprednisolone 2 mg/(kg·d) and ruxolitinib died. Watery diarrhea, increased levels of IL-5 and IL-8 and decreased IgM were more frequently in patients who did not survive.
ConclusionsFor children with fever, rash and a suspicious medication history, when complicated with hepatomegaly, impaired liver function and high levels of IL-5 and IL-8, DIHS-HLH should be considered. Once diagnosed with DIHS-HLH, suspicious drugs should be stopped immediately, and high dose intravenous immunoglobulin, corticosteroid and ruxolitinib could be used to control disease.
Key words:
Lymphohistiocytosis, hemophagocytic; Hypersensitivity; Child
Contributor Information
Zhao Yunze
Hematology Center, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China
Ma Honghao
Hematology Center, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China
Wang Dong
Hematology Center, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China
Lian Hongyun
Hematology Center, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China
Wang Tianyou
Hematology Center, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China
Zhang Rui
Hematology Center, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China
