儿童结内边缘区淋巴瘤

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  Paediatric noda lmarginal zone lymphoma
  儿童结内边缘区淋巴瘤
  Definition
  Paediatric nodal marginal zone lymphoma (NMZL) has distinctive clinical and morphological characteristics.
  儿童结内边缘区淋巴瘤(NMZL)具有独特的临床和形态学特征。
  It presents predominantly in males (with a male-to-female ratio of 20:1) with asymptomatic and localized disease (stage I in 90% of cases), mainly in the head and neck lymph nodes.
  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, it is similar to adult NMZL, except that there are often large follicles with extension of mantle zone B cells into the germinal centres, resembling progressively transformed germinal centres.
  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。
  The immunophenotype is similar to that of adult NMZL, with expansion of the interfollicular areas by CD20+ B cells that commonly coexpress CD43.
  免疫表型与成人NMZL相似,CD20阳性B细胞使滤泡间区扩大,这些CD20阳性B细胞常表达CD43。
  IgD staining may help to delineate an irregular and expanded mantle zone.
  IgD染色有助于勾勒出不规则扩张的套区。
  BCL2 is positive in half of the cases. CD10 is usually negative.
  半数病例BCL2阳性。CD10通常为阴性。
  Staining for CD279/PD1 shows numerous positive cells in the reactive germinal centres, a feature that may help in the differential diagnosis with paediatric-type follicular lymphoma, in which these cells are less numerous and pushed to the periphery of the germinal centre.
  CD279/PD1染色显示反应性生发中心有许多阳性细胞,这一特征可能有助于鉴别诊断儿童型滤泡性淋巴瘤(这些细胞数量较少,并被推到生发中心周围)。
  Clonal rearrangements of the IGHV region are detected in almost all cases. Trisomy 18 may be present in approximately one fifth of cases, and occasionally trisomy 3.
  几乎在所有病例中都能检测到IGHV区的克隆重排。约1/5病例存在18号染色体三体,偶尔出现3号染色体三体。
  The prognosis of paediatric NMZL is excellent, with a very low relapse rate and long survival following conservative treatment.
  儿童NMZL预后良好,保守治疗后复发率低,生存期长。
  The differential diagnosis with atypical marginal zone hyperplasia with monotypic immunoglobulin expression may be difficult, because the large cells in this condition also express CD43; although this type of hyperplasia has been reported in extranodal sites, some caution is advised because a similar process might also occur in the lymph nodes. Particularly for these reasons, genetic studies in paediatric marginal zone lymphomas are strongly recommended.
  与伴单型免疫球蛋白表达的不典型边缘区增生鉴别诊断可能很困难,因为这种情况下的大细胞也表达CD43;虽然这种增生报道是在结外,但应注意,因为在淋巴结中也可能发生类似的过程。特别是由于这些原因,强烈建议对儿童边缘区淋巴瘤进行遗传学研究。
  A marginal zone hyperplasia mimicking NMZL in head and neck lymph nodes of children has been associated with Haemophilus influenzae. The marginal zone cells in these cases are IgD-positive.
  与流感嗜血杆菌有关的儿童头颈淋巴结边缘区增生类似于NMZL。这些病例的边缘区细胞呈IgD阳性。
  ICD-O code
  9699/3
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