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

被引：0
|
作者：
Kurucu, Nilgun [1 ]
Kutluk, Tezer [1 ]
Saglam, Arzu [2 ]
Cagdas, Deniz [3 ]
Haliloglu, Mithat [4 ]
Salanci, Bilge Volkan [5 ]
Aydin, Burca [1 ]
Yalcin, Bilgehan [1 ]
Varan, Ali [1 ]
Uner, Aysegul [2 ]
机构：
[1] Hacettepe Univ, Fac Med & Canc Inst, Dept Pediat Oncol, Ankara, Turkiye
[2] Hacettepe Univ, Fac Med, Dept Pathol, Ankara, Turkiye
[3] Hacettepe Univ, Fac Med, Dept Pediat Immunol, Ankara, Turkiye
[4] Hacettepe Univ, Fac Med, Dept Pediat Radiol, Ankara, Turkiye
[5] Hacettepe Univ, Fac Med, Dept Nucl Med, Ankara, Turkiye
来源：
LEUKEMIA & LYMPHOMA | 2024年 / 65卷 / 12期
关键词：
Pediatric type follicular lymphoma; pediatric marginal zone lymphoma; pediatric indolent lymphomas; & imath; nborn errors of immunity; HEALTH-ORGANIZATION CLASSIFICATION; MARGINAL ZONE LYMPHOMA; FOLLICULAR LYMPHOMA; CHEMOTHERAPY;
D O I：
10.1080/10428194.2024.2374031
中图分类号：
R73 [肿瘤学];
学科分类号：
100214 ;
摘要：
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.
引用
收藏
页码：1875 / 1882
页数：8
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