Leukemia & lymphoma · 2024
Indolent B-cell non-Hodgkin lymphomas in children: high association with inborn errors of immunity.
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Kurucu Nilgün, Kutluk Tezer, Sağlam Arzu, Cagdas Deniz, Haliloğlu Mithat, Salancı Bilge Volkan, Aydın Burça, Yalçın Bilgehan, Varan Ali, Üner Ayşegül
Аннотация
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%) (p < 0.0001). Seventeen patients (85%) had stage I/II disease. Two patients received no treatment after surgery. Eleven patients were treated only with 3-6 courses of rituximab. Four patients received 3-6 courses of R-CHOP protocol. The prognosis was excellent Five years overall and event-free survivals were 100% and 85%, respectively.