OUTCOME IN CHILDREN WITH HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS TREATED USING HLH 2004 PROTOCOL IN NATIONAL CHILDREN HOSPITAL

Nguyễn Thị Hà1,, Nguyễn Thị Mai Hương1, Nguyễn Hoàng Nam1, Phạm Thị Hoài1, Trần Thị Mạnh1, Lưu Thị Nhàn1, Vũ Thị Bích Diệp1
1 Vietnam National Children's Hospital

Main Article Content

Abstract

Objective: To evaluate the effective treatment of HLH 2004 guideline in patients with hemophagocytic lymphohistiocytosis in national children hospital from 6/2016 to 5/ 2021. Method: We conducted a retrospective study in 122 HLH children who were hospitalized from 6/2016 to 5/ 2021 at Clinical Hemotology Department – National Children Hospital. All patients were treated with HLH- 2004 protocol. The outcome of patients was clasified into groups: non – active disease, active disease, reactivation of disease and death group. Results: A total of 122 patients were enrolled, with the rate fatality in the initial period was 12,3% (15/122), at the end of study was 28,7% (35/122). The 3 – year and 5 – year overall survival (OS) rate of all patients was 71,2% and 70,1%. The 3 – year and 5 – year event free survival (EFS) rate of all patients was 68,5% and 67,4%. Conclusion: OS rate was relatively high with 5 – year overall survival (OS) rate of all patients was 71,2% so we need to continue treating HLH- 2004 protocol early for HLH patient to reduce mortality rate.

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References

1. Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis.Annu Rev Med. 2012;63:233–46.
2. Henter JI, Elinder G, Soder O, et al. Incidence in Sweden and clinical features of familial hemophagocyticlymphohistiocytosis. Acta Paediatr Scand. 1991;80:428–35.
3. Nguyễn Thanh Liêm, Hồ Anh Tuấn, Nguyễn Văn Tú và cộng sự. Nghiên cứu đặc điểm lâm sàng, xét nghiệm và kết quả điều trị hội chứng thực bào máu. Tạp chí Nhi khoa.2007; 15(2): 11 – 15.
4. Lã Thị Bích Hồng. Nghiên cứu đặc điểm lâm sàng, cận lâm sàng và nhận xét kết quả điều trị hội chứng thực bào máu tại Bệnh viện Nhi Trung ương.Luận văn tốt nghiệp bác sỹ nội trú.2011 ; Đại học Y Hà Nội.
5. My. Comprehensive analyses and characterization of haemophagocytic lymphohistiocytosis in Viet Namese children.British Journal of Haematology. 2009;148: 301-310.
6. Elisabet Bergsten, AnnaCarin Horne, Maurizio Arico. Confirmed efficacy of etoposid and dexamethasone in HLH treatment: long – term results of the cooperative HLH – 2004 study. Blood.2017; Volume 130, N 25.
7. Ryu Yanagisawa, YozoNakazawa, Kazuyuki Matsuda. Outccome in children with hemophagocytic lymphohistiocytosis treated using HLH-2004 protocol in Japan. International Journal of Hemotology. 2019; 109: 206-213.
8. Tanusree Paul, Manas Kaira, Arun Danewa, et al. Pediatric hemophagocytic lymphohistiocytosis – A sing Center study of 118 patients. Indian pediatrics.2021; Vol.46
9. Li Xiao, Ying Xian, Bi-tao Dai, et al. Clinical features and outcome analysis of 83 childhood Epstein- Barr virus- associated hemophagocytic lymphohistiocytosis with HLH- 2004 protocol. Chin J Hematol.2011; Vol.32, No.10.
10. Trottestam H, Horne A, Arico M, et al. Chemo immune therapy for hemophagocytic lymphohistiocytosis: Long-term results of the HLH-94 treatment protocol. Blood.2011; 118:4577–4584. PMID:21900192