Lymphoma-based therapy for refractory or relapsed Epstein–Barr virus–related hemophagocytic lymphohistiocytosis in children
Epstein–Barr virus–related hemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening hyperinflammatory syndrome. Although etoposide-based immunochemotherapy has improved survival rates, consensus regarding the appropriate salvage therapy for patients with refractory or relapsed EBV-HLH is l...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-11-01
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| Series: | Journal of the Formosan Medical Association |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S0929664622000092 |
| _version_ | 1811240640496795648 |
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| author | Chao-Neng Cheng Wei-Ying Chu Kung-Chao Chang Jiann-Shiuh Chen |
| author_facet | Chao-Neng Cheng Wei-Ying Chu Kung-Chao Chang Jiann-Shiuh Chen |
| author_sort | Chao-Neng Cheng |
| collection | DOAJ |
| description | Epstein–Barr virus–related hemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening hyperinflammatory syndrome. Although etoposide-based immunochemotherapy has improved survival rates, consensus regarding the appropriate salvage therapy for patients with refractory or relapsed EBV-HLH is lacking. We performed a retrospective study to examine the efficacy of a lymphoma-based treatment regimen for children with refractory or relapsed EBV-HLH. The data of six children were analyzed. Four had cytogenetic abnormalities, and two experienced a transition to EBV-positive T-cell lymphoma. They were treated with an intensive chemotherapy regimen modified from that used in the Berlin–Frankfurt–Münster Group Trial as salvage therapy. Five patients (83%) achieved complete response. Four patients (67%) were disease free for a median of 10 years without undergoing allogeneic hematopoietic stem cell transplantation. No grade 3 or 4 nonhematologic adverse events occurred. Lymphoma-based chemotherapy is a potential curative treatment for some subgroups of children with refractory or relapsed EBV-HLH. |
| first_indexed | 2024-04-12T13:23:47Z |
| format | Article |
| id | doaj.art-3558e1f361324fb8b309bf23100478c1 |
| institution | Directory Open Access Journal |
| issn | 0929-6646 |
| language | English |
| last_indexed | 2024-04-12T13:23:47Z |
| publishDate | 2022-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of the Formosan Medical Association |
| spelling | doaj.art-3558e1f361324fb8b309bf23100478c12022-12-22T03:31:24ZengElsevierJournal of the Formosan Medical Association0929-66462022-11-011211123512355Lymphoma-based therapy for refractory or relapsed Epstein–Barr virus–related hemophagocytic lymphohistiocytosis in childrenChao-Neng Cheng0Wei-Ying Chu1Kung-Chao Chang2Jiann-Shiuh Chen3Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, TaiwanDepartment of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, TaiwanDepartment of Pathology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, TaiwanDepartment of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan; Corresponding author. Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, 138, Shengli Road, 704, Tainan, Taiwan.Epstein–Barr virus–related hemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening hyperinflammatory syndrome. Although etoposide-based immunochemotherapy has improved survival rates, consensus regarding the appropriate salvage therapy for patients with refractory or relapsed EBV-HLH is lacking. We performed a retrospective study to examine the efficacy of a lymphoma-based treatment regimen for children with refractory or relapsed EBV-HLH. The data of six children were analyzed. Four had cytogenetic abnormalities, and two experienced a transition to EBV-positive T-cell lymphoma. They were treated with an intensive chemotherapy regimen modified from that used in the Berlin–Frankfurt–Münster Group Trial as salvage therapy. Five patients (83%) achieved complete response. Four patients (67%) were disease free for a median of 10 years without undergoing allogeneic hematopoietic stem cell transplantation. No grade 3 or 4 nonhematologic adverse events occurred. Lymphoma-based chemotherapy is a potential curative treatment for some subgroups of children with refractory or relapsed EBV-HLH.http://www.sciencedirect.com/science/article/pii/S0929664622000092Epstein–Barr virusHemophagocytic lympho-histiocytosisRefractoryRelapseT-cell lymphoma |
| spellingShingle | Chao-Neng Cheng Wei-Ying Chu Kung-Chao Chang Jiann-Shiuh Chen Lymphoma-based therapy for refractory or relapsed Epstein–Barr virus–related hemophagocytic lymphohistiocytosis in children Journal of the Formosan Medical Association Epstein–Barr virus Hemophagocytic lympho-histiocytosis Refractory Relapse T-cell lymphoma |
| title | Lymphoma-based therapy for refractory or relapsed Epstein–Barr virus–related hemophagocytic lymphohistiocytosis in children |
| title_full | Lymphoma-based therapy for refractory or relapsed Epstein–Barr virus–related hemophagocytic lymphohistiocytosis in children |
| title_fullStr | Lymphoma-based therapy for refractory or relapsed Epstein–Barr virus–related hemophagocytic lymphohistiocytosis in children |
| title_full_unstemmed | Lymphoma-based therapy for refractory or relapsed Epstein–Barr virus–related hemophagocytic lymphohistiocytosis in children |
| title_short | Lymphoma-based therapy for refractory or relapsed Epstein–Barr virus–related hemophagocytic lymphohistiocytosis in children |
| title_sort | lymphoma based therapy for refractory or relapsed epstein barr virus related hemophagocytic lymphohistiocytosis in children |
| topic | Epstein–Barr virus Hemophagocytic lympho-histiocytosis Refractory Relapse T-cell lymphoma |
| url | http://www.sciencedirect.com/science/article/pii/S0929664622000092 |
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