Indolent B-cell non-Hodgkin lymphomas in children: high association with inborn errors of immunity

• Published in: Leukemia & lymphoma 2024-12, Vol.65 (12), p.1875-1882
• Main Authors: Kurucu, Nilgün, Kutluk, Tezer, Sağlam, Arzu, Cagdas, Deniz, Haliloğlu, Mithat, Salancı, Bilge Volkan, Aydın, Burça, Yalçın, Bilgehan, Varan, Ali, Üner, Ayşegül
• Format: Article
• Language: English
• Subjects: Adolescent; Antineoplastic Combined Chemotherapy Protocols - adverse effects; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Child; Child, Preschool; Cyclophosphamide - therapeutic use; Doxorubicin - therapeutic use; Female; Humans; Lymphoma, B-Cell - diagnosis; Lymphoma, B-Cell - drug therapy; Lymphoma, B-Cell - immunology; Lymphoma, B-Cell - pathology; Lymphoma, B-Cell - therapy; Lymphoma, B-Cell, Marginal Zone - diagnosis; Lymphoma, B-Cell, Marginal Zone - etiology; Lymphoma, B-Cell, Marginal Zone - pathology; Lymphoma, B-Cell, Marginal Zone - therapy; Male; Neoplasm Staging; Prednisone - therapeutic use; Prognosis; Retrospective Studies; Rituximab - therapeutic use; Treatment Outcome; Vincristine - therapeutic use
• ISSN: 1042-8194; 1029-2403; 1029-2403
• DOI: 10.1080/10428194.2024.2374031

Indolent lymphomas are rare in children and mostly consist of pediatric type follicular (PTFL) and pediatric marginal zone lymphomas (PMZL) and extranodal marginal zone lymphoma (ENMZL). Twenty children with indolent lymphoma (10 PTFL, 6 PMZL, 3 ENMZL, 1 mixed type) among 307 Non-Hodgkin Lymphoma (NHL) were retrospectively evaluated. The mean age of the entire group was 10.4 ± 4.4 and was significantly lower in PTFL than in PMZL. Seven patients (35%) had an associated inborn error of immunity (IEI) which was higher than that seen in aggressive lymphomas (5.9%) (