L-DEP regimen salvage therapy for refractory primary hemophagocytic lymphohistiocytosis triggered by Epstein-Barr virus infection in 4 children
10.3760/cma.j.cn112140-20240319-00188
- VernacularTitle:L-DEP方案挽救治疗难治性EB病毒感染诱发的原发性噬血细胞综合征4例
- Author:
Yunze ZHAO
1
;
Honghao MA
;
Hongyun LIAN
;
Dong WANG
;
Tianyou WANG
;
Rui ZHANG
Author Information
1. 国家儿童医学中心 首都医科大学附属北京儿童医院血液病中心 儿童血液病与肿瘤分 子分型北京市重点实验室 儿科学国家重点学科 儿科重大疾病研究教育部重点实验 室，北京 100045
- Keywords:
Lymphohistiocytosis, hemophagocytic;
Epstein-Barr virus infections;
Child;
Salvage therapy
- From:
Chinese Journal of Pediatrics
2024;62(5):467-472
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the efficacy and safety of the L-DEP regimen (asparaginase, liposome doxorubicin, etoposide and methylprednisolone) as a salvage therapy for the refractory primary hemophagocytic lymphohistocytosis triggered by Epstein-Barr virus infection (EBV-pHLH) in children.Methods:In this retrospective case study, clinical and laboratory data before and after L-DEP regimen of 4 children diagnosed with EBV-pHLH in Beijing Children′s hospital between January 2016 and June 2022 were collected, and the efficacy and safety of L-DEP regimen for the treatment of EBV-pHLH were analyzed.Results:Among 4 patients, there were 3 females and 1 male with the age ranged from 0.8 to 7.0 years. Two of them showed compound heterozygous mutations of PRF1, one with a heterozygous mutation of UNC13D, one homozygous mutation of ITK. Before the L-DEP therapy, all of them had anemia and a soaring level of soluble CD25, 3 patients had neutropenia and thrombopenia, 3 patients had a high level of ferritin, 3 patients had hypofibrinogenemia and 1 patient had hypertriglyceridemia. After receiving 1 or 2 cycles of L-DEP treatment, three achieved remission, including complete remission (1 case) and partial remission (2 cases), and the other one had no remission. The levels of blood cell counts, soluble CD25, triglyceride, fibrinogen and albumin were recovered gradually in 3 patients who got remission. All four patients underwent hematopoietic stem cell transplantation (HSCT) after L-DEP regimen, and three survived. All patients had no severe chemotherapy related complications. The main side effects were bone marrow suppression, infection and pancreatitis, which recovered after appropriate treatments, apart from one who died from severe infection after urgent HSCT.Conclusion:L-DEP regimen could be served as an effective and safe salvage treatment for refractory pediatric EBV-pHLH, and also provide an opportunity for patients to receive HSCT.
