A Rare Case of Early T-Precursor Lymphoblastic Lymphoma (ETP-LBL) in a Child With Nijmegen Breakage Syndrome.

IF 1.3 4区 医学 Q3 PATHOLOGY Pediatric and Developmental Pathology Pub Date : 2024-05-25 DOI:10.1177/10935266241255277
Kristina R Brannock, Samir B Kahwash
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Abstract

Lymphoblastic lymphoma (LBL) with an early T-cell precursor phenotype has only been rarely reported. Nijmegen breakage syndrome (NBS) is an inherited chromosomal instability disorder with known predisposition to malignancies that is very rare as well. We report a case of early T-precursor LBL (ETP-LBL) in a patient with NBS, a rare combination that has not been reported. We raise the question of whether a chromosomal instability disorder such as NBS increases the propensity for early T-precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL), given that ETP-ALL has been shown to have increased genomic instability compared to T-ALL.

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奈梅亨断裂综合征患儿罕见的早期 T 前体淋巴母细胞淋巴瘤(ETP-LBL)病例。
具有早期T细胞前体表型的淋巴母细胞淋巴瘤(LBL)鲜有报道。奈梅亨断裂综合征(NBS)是一种遗传性染色体不稳定疾病,具有已知的恶性肿瘤易感性,但也非常罕见。我们报告了一例 NBS 患者的早期 T 前体 LBL(ETP-LBL)病例,这种罕见的合并症尚未见报道。鉴于 ETP-ALL 与 T-ALL 相比具有更高的基因组不稳定性,我们提出了这样一个问题:NBS 等染色体不稳定性疾病是否会增加早期 T 前体急性淋巴细胞白血病/淋巴瘤(ETP-ALL/LBL)的发病倾向?
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来源期刊
CiteScore
3.70
自引率
5.30%
发文量
59
审稿时长
6-12 weeks
期刊介绍: The Journal covers the spectrum of disorders of early development (including embryology, placentology, and teratology), gestational and perinatal diseases, and all diseases of childhood. Studies may be in any field of experimental, anatomic, or clinical pathology, including molecular pathology. Case reports are published only if they provide new insights into disease mechanisms or new information.
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