Pediatric gray zone lymphoma according to the 2022 WHO classification : An Italian cohort study
© 2023 Wiley Periodicals LLC..
BACKGROUND: The 2022 World Health Organization (WHO) classification redefines the concept of gray zone lymphoma (GZL), restricting it in practice to cases of mediastinal/thymic origin (mediastinal gray zone lymphoma, MGZL) with overlapping features between primary mediastinal B-cell lymphoma (PMBCL) and classical Hodgkin lymphoma (CHL). Cases with histological characteristics of GZL but occurring without mediastinal involvement are better classified as diffuse large B-cell lymphoma, not otherwise specified (DLBCL NOS), with few exceptions.
PROCEDURE: We collected clinical and pathological data about all Italian pediatric patients diagnosed with GZL over a 20-year period.
RESULTS: We identified only four cases of bona fide MGZL. All patients were adolescent and presented with a mediastinal disease, always associated with other nodal involvement. B symptoms and increased levels of both erythrocyte sedimentation rate (ESR) and lactate dehydrogenase (LDH) were observed. Only two patients achieved a first complete remission, suggesting a more aggressive clinical behavior than either PMBCL or CHL.
CONCLUSION: Prospective studies evaluating prognostic factors and establishing the most effective first-line therapy for MGZL are highly needed.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - year:2023 |
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| Enthalten in: |
Pediatric blood & cancer - (2023) vom: 30. Mai, Seite e30481 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Restivo, Giulia Angela [VerfasserIn] |
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| Links: |
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| Themen: |
Gray zone lymphoma |
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| Anmerkungen: |
Date Revised 31.05.2023 published: Print-Electronic Citation Status Publisher |
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| doi: |
10.1002/pbc.30481 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM357566203 |
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| 520 | |a © 2023 Wiley Periodicals LLC. | ||
| 520 | |a BACKGROUND: The 2022 World Health Organization (WHO) classification redefines the concept of gray zone lymphoma (GZL), restricting it in practice to cases of mediastinal/thymic origin (mediastinal gray zone lymphoma, MGZL) with overlapping features between primary mediastinal B-cell lymphoma (PMBCL) and classical Hodgkin lymphoma (CHL). Cases with histological characteristics of GZL but occurring without mediastinal involvement are better classified as diffuse large B-cell lymphoma, not otherwise specified (DLBCL NOS), with few exceptions | ||
| 520 | |a PROCEDURE: We collected clinical and pathological data about all Italian pediatric patients diagnosed with GZL over a 20-year period | ||
| 520 | |a RESULTS: We identified only four cases of bona fide MGZL. All patients were adolescent and presented with a mediastinal disease, always associated with other nodal involvement. B symptoms and increased levels of both erythrocyte sedimentation rate (ESR) and lactate dehydrogenase (LDH) were observed. Only two patients achieved a first complete remission, suggesting a more aggressive clinical behavior than either PMBCL or CHL | ||
| 520 | |a CONCLUSION: Prospective studies evaluating prognostic factors and establishing the most effective first-line therapy for MGZL are highly needed | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a WHO classification | |
| 650 | 4 | |a gray zone lymphoma | |
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| 700 | 1 | |a Farruggia, Piero |e verfasserin |4 aut | |
| 700 | 1 | |a Pillon, Marta |e verfasserin |4 aut | |
| 700 | 1 | |a Mascarin, Maurizio |e verfasserin |4 aut | |
| 700 | 1 | |a Elia, Caterina |e verfasserin |4 aut | |
| 700 | 1 | |a Recupero, Santina |e verfasserin |4 aut | |
| 700 | 1 | |a Muggeo, Paola |e verfasserin |4 aut | |
| 700 | 1 | |a Cagnazzo, Celeste |e verfasserin |4 aut | |
| 700 | 1 | |a Onofrillo, Daniela |e verfasserin |4 aut | |
| 700 | 1 | |a Mura, Rosamaria |e verfasserin |4 aut | |
| 700 | 1 | |a Furfari, Ilaria |e verfasserin |4 aut | |
| 700 | 1 | |a Trizzino, Angela |e verfasserin |4 aut | |
| 700 | 1 | |a Bertolini, Patrizia |e verfasserin |4 aut | |
| 700 | 1 | |a Sala, Alessandra |e verfasserin |4 aut | |
| 700 | 1 | |a De Santis, Raffaela |e verfasserin |4 aut | |
| 700 | 1 | |a Galimberti, Daniela |e verfasserin |4 aut | |
| 700 | 1 | |a Schiavello, Elisabetta |e verfasserin |4 aut | |
| 700 | 1 | |a Carraro, Elisa |e verfasserin |4 aut | |
| 700 | 0 | |a Emanuele Stefano Giovanni D'Amore |e verfasserin |4 aut | |
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