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Abstract
Background: Hemophagocytic lymphohistiocytosis (HLH) is a life - threatening condition caused by excessive immune system activation. HLH can be primary or secondary. Secondary HLH is often triggered by viral infection, malignancy or rheumatoid disease. Secondary HLH associated with malignancy is common in adults,
but very rare in children. To date, only a few cases of malignancy - associated HLH in children have been reported.
Case report: We report a 4 - year - old boy, diagnosed and treated for B-cell acute lymphoblastic leukemia, who achieved complete remission, undergoing maintenance
chemotherapy. The child presented with persistently high fever despite antibiotic therapy, laboratory tests met criteria for HLH and high H-score suggest HLH. The child was treated with Dexamethasone alone according to the 8-week regimen, the children responded well. He have been currently receiving chemotherapy in the maintenance phase.
Conclusion: HLH in malignant disease in pediatrics remains a challenge that has substantial mortality. Treatment decisions must be taken case - by - case. Larger
studies are needed to improve disease awareness and prognosis.
Keywords: Hemophagocytic lymphohistiocytosis, malignancy - associated hemophagocytic lymphohistiocytosis, child, cancer.
References
Bộ y tế. Hội chứng thực bào máu. Hướng dẫn chẩn đoán và điều trị một số bệnh thường gặp ở trẻ em, Bộ y tế, Quyết định số 3312/QĐ-BYT ngày 07/8/2015; 2015: 564-571.
Naval Daver, Kenneth McClain, Carl E. Allen, Sameer A. Parikh, Zaher Otrock, Cristhiam Rojas-Hernandez, Boris Blechacz, Sa Wang, Milen Minkov, Michael B. Jordan, Paul La Rosée, Hagop M. Kantarjian. A consensus review on malignancy-associated hemophagocytic
lymphohistiocytosis in adults. Cancer 2017; 123 (17), 3229-3240.
France Debaugnies, Bhavna Mahadeb, Alina Ferster, et al. Performances of the H-Score for Diagnosis of Hemophagocytic Lymphohistiocytosis in Adult and Pediatric Patients. Am J Clin Pathol 2016; 145, 862-870.
Laurence Fardet, Lionel Galicier, Olivier Lambotte, et al. Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome. Arthritis & Rheumatology 2014; 66 (9), 2613-2620.
James W. Farquhar, Albert E. Claireaux. Familial Haemophagocytic Reticulosis. Archives of Disease in Childhood 1952; 27 (136), 519-525.
Jan-Inge Henter, AnnaCarin Horne, Maurizio Aricó, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer 2007; 48 (2), 124-131.
Zhizhuo Huang, Yueping Jia, Yingxi Zuo, Jun Wu, Aidong Lu, Leping Zhang. Malignancy-associated hemophagocytic lymphohistiocytosis in children: a 10-year experience of a single pediatric hematology center. Hematology 2020; 25 (1), 389-399.
Kai Lehmberg, Björn Sprekels, Kim E. Nichols, et al. Malignancy - associated haemophagocytic lymphohistiocytosis in children and adolescents. British Journal of Haematology 2015; 170 (4), 539-549.
Hua Pan, Yongmin Huo, Lirong Sun. Comparison between clinical features and prognosis of malignancy- and nonmalignancy–associated pediatric hemophagocytic lymphohistiocytosis. BMC Pediatrics 2019; 19 (1), 468.
Nattapol Rungrojjananon, Samart Pakakasama, Angkana Winaichatsak, et al. Pediatric hemophagocytic lymphohistiocytosis in a tropical country: Results of a multicenter study in Thailand. Asia-Pacific Journal of Clinical Oncology 2022; n/a (n/a)
Amitabh Singh, Lesa Dawman, Rachna Seth. Malignancy associated hemophagocytic lymphohistiocytosis in children. Journal of Cancer Research and Therapeutics 2018; 14 (3), 559-562.
Mourad Tiab, Françoise Mechinaud, Jean-Luc Harousseau. Haemophagocytic syndrome associated with infections. Best Practice & Research Clinical Haematology 2000; 13 (2), 163-178.
K. Lehmberg, K. E. Nichols, J. I. Henter, et al. Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies. Haematologica 2015; 100 (8), 997-1004.
C. Shimazaki, T. Inaba, M. Nakagawa. B-cell lymphomaassociated hemophagocytic syndrome. Leuk Lymphoma 2000; 38 (1-2), 121-30.
V. Strenger, G. Merth, H. Lackner, et al. Malignancy and chemotherapy induced haemophagocytic lymphohistiocytosis in children and adolescents-a single centre experience of 20 years. Ann Hematol 2018; 97 (6), 989-998.
T. Tsuji, T. Hirano, H. Yamasaki, M. Tsuji, H. Tsuda. A high sIL-2R/ferritin ratio is a useful marker for the diagnosis of lymphoma-associated hemophagocytic syndrome. Ann Hematol 2014; 93 (5), 821-6.
N. Takahashi, A. Nakahachi. [Clinicopathological characteristics of adult patients with lymphoma-associated hemophagocytic syndrome in Japan]. Rinsho Ketsueki 1999; 40 (2), 96-8
| Published | 12-01-2025 | |
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| Issue | No. 85 (2023) | |
| Section | Case report | |
| DOI | 10.38103/jcmhch.85.21 | |
| Keywords | Từ khóa: Hội chứng thực bào máu, Hội chứng thực báo máu liên quan bệnh lý ác tính, trẻ em, ung thư. Keywords: Hemophagocytic lymphohistiocytosis, malignancy - associated hemophagocytic lymphohistiocytosis, child, cancer. |
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