Pediatric gray zone lymphoma according to the 2022 WHO classification : An Italian cohort study
Abstract 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 - volume:70 |
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| Enthalten in: |
Pediatric Blood & Cancer - 70(2023), 9 |
| Beteiligte Personen: |
Restivo, Giulia Angela [VerfasserIn] |
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| Anmerkungen: |
© 2023 Wiley Periodicals LLC. |
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| Umfang: |
7 |
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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): |
WLY016556933 |
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| 520 | |a Abstract 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. | ||
| 700 | 1 | |a Farruggia, Piero |4 aut | |
| 700 | 1 | |a Pillon, Marta |4 aut | |
| 700 | 1 | |a Mascarin, Maurizio |4 aut | |
| 700 | 1 | |a Elia, Caterina |4 aut | |
| 700 | 1 | |a Recupero, Santina |4 aut | |
| 700 | 1 | |a Muggeo, Paola |4 aut | |
| 700 | 1 | |a Cagnazzo, Celeste |4 aut | |
| 700 | 1 | |a Onofrillo, Daniela |4 aut | |
| 700 | 1 | |a Mura, Rosamaria |4 aut | |
| 700 | 1 | |a Furfari, Ilaria |4 aut | |
| 700 | 1 | |a Trizzino, Angela |4 aut | |
| 700 | 1 | |a Bertolini, Patrizia |4 aut | |
| 700 | 1 | |a Sala, Alessandra |4 aut | |
| 700 | 1 | |a De Santis, Raffaela |4 aut | |
| 700 | 1 | |a Galimberti, Daniela |4 aut | |
| 700 | 1 | |a Schiavello, Elisabetta |4 aut | |
| 700 | 1 | |a Carraro, Elisa |4 aut | |
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