儿童结内边缘区淋巴瘤

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Paediatricnodal

marginalzonelymphoma

儿童结内边缘区淋巴瘤

Definition

Paediatricnodalmarginalzonelymphoma(NMZL)hasdistinctiveclinicalandmorphologicalcharacteristics.

儿童结内边缘区淋巴瘤（NMZL）具有独特的临床和形态学特征。

Itpresentspredominantlyinmales(withamale-to-femaleratioof20:1)withasymptomaticandlocalizeddisease(stageIin90%ofcases),mainlyintheheadandnecklymphnodes.

好发于男性，男女比例为20:1；主要发生于头颈部淋巴结，表现为无症状的局限性病变（90%病例临床分期为I期）。

Histologically,itissimilartoadultNMZL,exceptthatthereareoftenlargefollicleswithextensionofmantlezoneBcellsintothegerminalcentres,resemblingprogressivelytransformedgerminalcentres.

组织学上，它与成人NMZL相似，只是通常有大的滤泡，套区B细胞延伸到生发中心，类似于进行性生发中心转化。

TheimmunophenotypeissimilartothatofadultNMZL,withexpansionoftheinterfollicularareasbyCD20+Bcellsthat