Pediatric gray zone lymphoma according to the 2022 WHO classification: An Italian cohort study.
Pediatr Blood Cancer
; : e30481, 2023 May 30.
Article
en En
| MEDLINE
| ID: mdl-37254478
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.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Tipo de estudio:
Etiology_studies
/
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Idioma:
En
Revista:
Pediatr Blood Cancer
Asunto de la revista:
HEMATOLOGIA
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NEOPLASIAS
/
PEDIATRIA
Año:
2023
Tipo del documento:
Article
País de afiliación:
Italia