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

IF 2.2 4区 医学 Q3 HEMATOLOGY Leukemia & Lymphoma Pub Date : 2024-12-01 Epub Date: 2024-07-05 DOI:10.1080/10428194.2024.2374031
Nilgün Kurucu, Tezer Kutluk, Arzu Sağlam, Deniz Cagdas, Mithat Haliloğlu, Bilge Volkan Salancı, Burça Aydın, Bilgehan Yalçın, Ali Varan, Ayşegül Üner
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Abstract

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.

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儿童惰性 B 细胞非霍奇金淋巴瘤:与先天性免疫错误关系密切。
淋巴瘤在儿童中非常罕见,主要包括小儿滤泡型淋巴瘤(PTFL)、小儿边缘区淋巴瘤(PMZL)和结节外边缘区淋巴瘤(ENMZL)。研究人员对307例非霍奇金淋巴瘤(NHL)患儿中的20例非霍奇金淋巴瘤患儿(10例PTFL、6例PMZL、3例ENMZL、1例混合型)进行了回顾性评估。全组患者的平均年龄为(10.4 ± 4.4)岁,PTFL患者的平均年龄明显低于PMZL患者。7名患者(35%)伴有先天性免疫错误(IEI),高于侵袭性淋巴瘤(5.9%)(P<0.05)。
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来源期刊
Leukemia & Lymphoma
Leukemia & Lymphoma 医学-血液学
CiteScore
4.10
自引率
3.80%
发文量
384
审稿时长
1.8 months
期刊介绍: Leukemia & Lymphoma in its fourth decade continues to provide an international forum for publication of high quality clinical, translational, and basic science research, and original observations relating to all aspects of hematological malignancies. The scope ranges from clinical and clinico-pathological investigations to fundamental research in disease biology, mechanisms of action of novel agents, development of combination chemotherapy, pharmacology and pharmacogenomics as well as ethics and epidemiology. Submissions of unique clinical observations or confirmatory studies are considered and published as Letters to the Editor
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