Aqil Barina, Merritt Brian Y, Elghetany M Tarek, Kamdar Kala Y, Lu Xinyan Y, Curry Choladda V
1 Department of Pathology & Immunology, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA.
Pediatr Dev Pathol. 2015 Mar-Apr;18(2):167-71. doi: 10.2350/14-12-1584-CR.1. Epub 2015 Jan 27.
Nodal marginal zone lymphoma (NMZL) is a B-cell lymphoma that shares morphologic and immunophenotypic features with extranodal and splenic marginal zone lymphomas but lacks extranodal or splenic involvement at presentation. NMZL occurs mostly in adults with no sex predilection, at advanced stage (III or IV), with frequent relapses and a high incidence of tumoral genetic abnormalities including trisomies 3 and 18 and gain of 7q. Pediatric NMZL, however, is a rare but distinct variant of NMZL with characteristic features including male predominance, asymptomatic and localized (stage I) disease, low relapse rates with excellent outcomes, and a lower incidence of essentially similar genetic aberrations compared to adult NMZL. Here we describe a unique case of childhood NMZL with unusual clinicopathologic features for the pediatric variant including generalized lymphadenopathy, high-stage disease with persistence after therapy, unusual immunophenotype (CD5, CD23, and BCL6 positive), and unique chromosomal abnormalities including monosomy 20 and add(10)(p11.2).
结外边缘区淋巴瘤（NMZL）是一种B细胞淋巴瘤，它与结外和脾边缘区淋巴瘤具有形态学和免疫表型特征，但在初诊时无结外或脾脏受累。NMZL主要发生于成年人，无性别倾向，处于晚期（III或IV期），常有复发，且肿瘤基因异常发生率高，包括3号和18号染色体三体以及7q获得。然而，儿童NMZL是NMZL的一种罕见但独特的变异型，其特征包括男性居多、无症状且局限于局部（I期）疾病、复发率低且预后良好，与成人NMZL相比，基本相似的基因畸变发生率较低。在此，我们描述了一例儿童NMZL的独特病例，其具有小儿变异型不寻常的临床病理特征，包括全身淋巴结肿大、治疗后持续存在的晚期疾病、不寻常的免疫表型（CD5、CD23和BCL6阳性）以及独特的染色体异常，包括20号染色体单体和add(10)(p11.2)。
