淋巴结边缘区淋巴瘤 (NMZL) 是一种罕见的非霍奇金淋巴瘤 (NHL)，约占儿科和成人所有 NHL 的 2%。它被认为是边缘区淋巴瘤最不常见的亚型，在儿科中很少见，通常被视为轻度疾病。在成人中，其表现从轻微到侵袭性疾病不等，伴有多处淋巴结肿大和全身症状。在这里，我们报告了一个有趣的病例，一名 31 个月大的男孩出现了难治性 EBV 阳性 NMZL，并伴有广泛的疾病。该肿瘤对常规化疗和利妥昔单抗反应不佳。因此，我们通过二线化疗（包括硼替佐米）治疗该疾病，然后进行同种异体造血干细胞移植（HSCT），取得了良好的结果。据报道，侵袭性 NMZL 儿童的病例很少。该肿瘤对一线化疗耐药。然而，通过同种异体干细胞移植联合清髓性预处理化疗取得了成功的结果。这一特殊病例的意义在于，这是儿科报道的第一例难治性 NMZL 病例，该病例对同种异体干细胞移植反应良好。© 2024 Wiley periodicals LLC。

Nodal marginal zone lymphoma (NMZL) is a rare form of non-Hodgkin lymphoma (NHL), accounting for around 2% of all NHL in pediatrics and adults. It is considered the least common subtype of marginal zone lymphoma, which is rarely seen in pediatrics and is usually viewed as a mild disease. In adults, its presentation varies from mild to aggressive disease with multiple lymph node enlargement and systemic symptoms.Here, we report an interesting case of a 31-month-old boy who developed refractory EBV-positive NMZL with extensive disease. The tumor did not respond well to conventional chemotherapy and Rituximab. Therefore, we manage the disease with second-line chemotherapy, including bortezomib, followed by an allogeneic hematopoietic stem cell transplant (HSCT) with an excellent outcome.Very few cases have been reported for aggressive NMZL children. The tumor was refractory to first line chemotherapy. However, the successful outcome was achieved by allogeneic stem cell transplant with myeloablative conditioning chemotherapy. The significance of this particular case is that this is the first reported case from pediatrics for refractory NMZL, which responded well to allogeneic stem cell transplant.© 2024 Wiley Periodicals LLC.