CAJ | 학술논문

目的：探讨血管免疫母细胞性T细胞淋巴瘤伴霍奇金样细胞的临床表现、病理诊断与鉴别诊断。方法对6例血管免疫母细胞性T细胞淋巴瘤伴霍奇金样细胞进行形态学观察、免疫组织化学EliVision法及EB病毒编码的小RNA（EBER）原位杂交技术分析，并复习文献。结果形态学特点为淋巴结结构破坏，多形性淋巴细胞浸润，在嗜酸性粒细胞、组织细胞、浆细胞的炎症背景下，散在数量不等的大细胞，间质小血管增生明显。部分大细胞形态似RS细胞，单核、多核，嗜酸性核仁明显。免疫组织化学染色显示：CD3、CD5广泛阳性，CD10、bcl-6部分细胞阳性，CD20灶状及散在阳性， CD21显示增多、紊乱的树状突细胞网（ FDC）围绕血管生长。其中霍奇金样大细胞CD20阳性或弱阳性，PAX-5弱阳性或阳性，CD30灶性强弱不等阳性或强阳性，CD15阴性，MUM-1强阳性或阳性，Ki-67阳性。 EBER特点为大、中、小细胞散在阳性或簇状阳性，其中霍奇金样细胞阳性。结论血管免疫母细胞性T细胞淋巴瘤伴霍奇金样细胞是侵袭性淋巴瘤，形态学与经典型霍奇金淋巴瘤存在交叉， EBER、免疫表型较相似，容易误诊，需结合临床表现、组织学形态、免疫表型、EBER乃至基因重排综合分析、诊断。
목적：탐토혈관면역모세포성T세포림파류반곽기금양세포적림상표현、병리진단여감별진단。방법대6례혈관면역모세포성T세포림파류반곽기금양세포진행형태학관찰、면역조직화학EliVision법급EB병독편마적소RNA（EBER）원위잡교기술분석，병복습문헌。결과형태학특점위림파결결구파배，다형성림파세포침윤，재기산성립세포、조직세포、장세포적염증배경하，산재수량불등적대세포，간질소혈관증생명현。부분대세포형태사RS세포，단핵、다핵，기산성핵인명현。면역조직화학염색현시：CD3、CD5엄범양성，CD10、bcl-6부분세포양성，CD20조상급산재양성， CD21현시증다、문란적수상돌세포망（ FDC）위요혈관생장。기중곽기금양대세포CD20양성혹약양성，PAX-5약양성혹양성，CD30조성강약불등양성혹강양성，CD15음성，MUM-1강양성혹양성，Ki-67양성。 EBER특점위대、중、소세포산재양성혹족상양성，기중곽기금양세포양성。결론혈관면역모세포성T세포림파류반곽기금양세포시침습성림파류，형태학여경전형곽기금림파류존재교차， EBER、면역표형교상사，용역오진，수결합림상표현、조직학형태、면역표형、EBER내지기인중배종합분석、진단。
Objective To study the clinicopathologic features and pathologic diagnosis and differential diagnosis of angioimmunoblastic T-cell lymphoma with HRS-like cells.Methods Six cases of angioimmunoblastic T-cell lymphoma with HRS-like cells were examined histologically and immunohistochemically ( EliVision method ) and in-situ hybridization for Epstein-Barr virus-encoded RNA (EBER), and the literature was reviewed.Results The cytologic and microscopic features of these imprints and lymph node samples showed a heterogeneous population of hematolymphoid cells , including small to intermediate lymphoid cells, immunoblasts, plasma cells, dendritic cells, and eosinophils, as well as small vessels that were surrounded by some of the abnormal cells.The neoplastic T-cells expressed CD3 and CD5 and partly positive for CD10 and bcl-6, CD21 showed expanded and irregular follicular dendritic cell ( FDC) meshworks that surrounding the high HEV.The HRS-like cells were positive for MUM-1 and Ki-67, variable intensity positive for CD30, CD20, and PAX-5, but negative for CD15.EBV-positive cells included HRS-like cells and small to large-sized neoplastic T-cells, which formed small clusters or scattering in the background of the disease.Conclusions The clinical course of angioimmunoblastic T-cell lymphoma with HRS-like cells is aggressive.Which present with histomorphology overlap with classical Hodgkin lymphoma ( CHL) , similar to CHL in EBER and immunophenotype , however, it is easy to misdiagnosis as HL.Thus, angioimmunoblastic T-cell lymphoma pathology diagnosis should comprehensive analysis of different kinds of materials , including clinical features , and histological structure , and EBER , and immunophenotype , and gene rearrangement .