ALK阳性的弥漫性大B细胞淋巴瘤

ALK-positive diffuse large B cell lymphoma

目的 探讨间变性淋巴瘤激酶(ALK)阳性的弥漫性大B细胞淋巴瘤的组织病理形态和免疫组化表达的意义。方法 参照WHO2001年恶性淋巴瘤分类,对222例弥漫性大B细胞淋巴瘤进行形态学观察和免疫组化Polymer两步法标记。结 果 6例弥漫性大B细胞淋巴瘤免疫组化ALK阳性表达,阳性反应物质定位于细胞质内,成粗大的颗粒状,1例合并CD30阳 性表达,全部表达B系列抗原CD20、CD79α和CD138,4例不表达CD45。组织病理形态:3例为浆母细胞性,2例为免疫母细 胞性伴浆细胞样分化,1例为间变性。结论 ALK阳性的弥漫性大B细胞淋巴瘤是组织形态和免疫表型独特的变异类型,与 CLTC- ALK基因易位和NPM- ALK融合基因易位有关,其分子遗传学的异质性不同于以往的认识。

Purpose To explore the significance of diffuse large B-cell lymphoma ( DLBCL) with a granular cytoplasmic staining for anaplastic lymphoma kinase (ALK) and their morphologic variants. Methods 222 cases of DLBCL based on morphology were reviewed by 2001 WHO classification of HL and immunohistochemical staining for CD20,CD79α,CD3,CD45RO,CD45,CD5,CD30,CD138,ALK,bcl-2,CD10,bcl-6,EMA,TIA1,κ/λ were carried out with the Polymer 2-step method. Result Immunostaining showed 6 cases were characterized by a granular cytoplasmic staining for ALK as well as express various pan-B markers shch as CD20,CD79 and CD138,but lack CD45 in 4 cases. One anaplastic case co-expressed ALK and CD30. Histopathologic morphogy presented as plasmablastic,immunoblastic with plasmacytoid differentiation and anaplastic. Conclusions ALK-positive DLBCL is a immunophenotypically and morphologically distinctive variant of diffuse large B-cell lymphoma,which is close correlation with CLTC-ALK rearrangement and NPM-ALK rearrangement and more hererogeneous at the molecular level than previously recognized.