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 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..
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:121 |
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| Enthalten in: |
Journal of the Formosan Medical Association - 121(2022), 11, Seite 2351-2355 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Chao-Neng Cheng [VerfasserIn] |
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| Links: |
doi.org [kostenfrei] |
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| Themen: |
Epstein–Barr virus |
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| doi: |
10.1016/j.jfma.2022.01.009 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
DOAJ021350809 |
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| 520 | |a 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. | ||
| 650 | 4 | |a Epstein–Barr virus | |
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