Taddesse-Heath Lekidelu, Pittaluga Stefania, Sorbara Lynn, Bussey Mary, Raffeld Mark, Jaffe Elaine S
Hemopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute/NIH, 10 Center Drive, Bethesda, MD 20892, USA.
Am J Surg Pathol. 2003 Apr;27(4):522-31. doi: 10.1097/00000478-200304000-00014.
We describe the clinicopathologic findings of 48 cases of marginal zone B-cell lymphoma (MZL) in children and young adults, a disease that has been recognized previously only rarely in this age group. Patients ranged in age from 2 to 29 years, with pediatric patients (< or =18 years) comprising 52% of the cases. As in adults, both primary nodal (N) and extranodal (E) MZL were observed. However, primary NMZL comprised the majority of the cases (67%) and demonstrated distinctive clinical and histologic features. NMZL occurred most commonly in young males (median 16 years, male/female ratio 5.4:1), with no underlying disease, presenting as localized adenopathy (90% stage I), with excellent prognosis and low rate of recurrence. In contrast, EMZL were much less common, and patients were older (median 24.5 years), with only a slight male predominance (male/female ratio 1.2:1). Most patients had localized disease (73% stage I) with excellent prognosis and infrequent recurrences. In addition, an association with autoimmune disease was observed in 19% of the EMZL. Both primary NMZL and EMZL in young patients shared similar morphologic and immunophenotypic findings to those described in adults and were monoclonal B-cell proliferations with monoclonality demonstrated in 94% of the cases. A common morphologic feature in NMZL was disruption of residual follicles resembling progressive transformation of germinal centers (PTGC), observed in 66% of the cases. Although the precise relationship of primary NMZL and the PTGC-like changes is unclear, it is possible that NMZL arises in a background of PTGC, as florid PTGC often occurs in young males. We conclude that EMZL in children and young adults are similar to EMZL of mucosa-associated lymphoma tissue occurring in older patients. However, pediatric NMZL appear to have distinctive clinical and histologic features.
我们描述了48例儿童和青年边缘区B细胞淋巴瘤（MZL）的临床病理特征，该病此前在该年龄组中仅罕见报道。患者年龄范围为2至29岁，其中儿科患者（≤18岁）占病例的52%。与成人一样，观察到原发性淋巴结（N）和结外（E）MZL。然而，原发性N MZL占大多数病例（67%），并表现出独特的临床和组织学特征。N MZL最常发生于年轻男性（中位年龄16岁，男女比例5.4:1），无基础疾病，表现为局限性淋巴结肿大（90%为I期），预后良好，复发率低。相比之下，E MZL则少见得多，患者年龄较大（中位年龄24.5岁），男性略占优势（男女比例1.2:1）。大多数患者有局限性疾病（73%为I期），预后良好，复发罕见。此外，19%的E MZL患者与自身免疫性疾病有关。年轻患者的原发性N MZL和E MZL在形态学和免疫表型上与成人中描述的相似，均为单克隆B细胞增殖，94%的病例显示单克隆性。N MZL的一个常见形态学特征是残留滤泡破坏，类似于生发中心进行性转化（PTGC），66%的病例观察到该特征。虽然原发性N MZL与PTGC样改变的确切关系尚不清楚，但N MZL可能起源于PTGC背景，因为 florid PTGC常发生于年轻男性。我们得出结论，儿童和青年的E MZL与老年患者发生的黏膜相关淋巴瘤组织的E MZL相似。然而，儿科N MZL似乎具有独特的临床和组织学特征。
