奈梅亨断裂综合征儿童早期 T 前体淋巴母细胞淋巴瘤 (ETP-LBL) 的罕见病例。
A Rare Case of Early T-Precursor Lymphoblastic Lymphoma (ETP-LBL) in a Child With Nijmegen Breakage Syndrome.
发表日期：2024 May 25
作者：
Kristina R Brannock, Samir B Kahwash
来源：
MOLECULAR & CELLULAR PROTEOMICS
摘要：
具有早期 T 细胞前体表型的淋巴母细胞淋巴瘤 (LBL) 的报道很少。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倾向，但也非常罕见。我们报告了一例 NBS 患者出现早期 T 前体 LBL (ETP-LBL) 的病例，这是一种尚未报道的罕见组合。我们提出这样的问题：染色体不稳定疾病（例如 NBS）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) 的倾向，因为与 T 相比，ETP-ALL 已被证明具有更高的基因组不稳定性。 -全部。
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.