淋巴结血管内T细胞淋巴瘤1例报道及文献复习

目的　探讨血管内淋巴瘤 (IVL)的临床病理特征。方法　对 1例腹股沟淋巴结IVL临床、病理组织学及免疫表型进行观察分析并复习文献。结果　男性 31岁 ,不明原因高热伴消瘦 5 0天 ,右腹股沟直径 1cm淋巴结 1枚 ,B超示肝脏轻度增大 ,血LDH明显升高伴ESR及转氨酶轻度升高 ,外周血WBC 3 3× 10 7/L ,骨髓像、多种病原及各肿瘤相关抗原检测均无异常。病理活检 :腹股沟淋巴结大部分破坏 ,代之以大量扩张的中小血管 ,腔内充满大量异型淋巴样细胞 ,局部伴管壁、管周浸润并累及结外脂肪组织。瘤细胞免疫表型CD4 5、CD4 5RO、CD3阳性 ,CK、CD6 8、CD79α、CD2 0均阴性 ,血管壁及内皮细胞CD31、CD34阳性。行CHOP化疗后症状缓解 ,现仍在随访中。结论　IVL是一罕见的非霍奇金淋巴瘤 ,好发于中枢神经系统及皮肤 ,其他部位少见 ,绝大数为B细胞型 ,T型罕见 ,以浅表淋巴结活检确诊者尚无报道。临床表现有一定提示性 ,确诊靠组织病理学检查 ,部分病例对化疗敏感 ,但多数病例预后差

Intravascular lymphoma of lymph node:a case report and review of the literature

Purpose To investigate the clinical and pathological features of intravascular lymphoma (IVL). Methods A case of IVL in inguinal lymph node was studied with clinical findings,histopathology and immunophenotype as well as literature review. Results A case of 31 year old man presented with unknown fever and weight loss for 50 days, one of the right inguinal lymph node was found, liver was light enlargement with elevation of serum LDH, GOP, GTP and ESR. Leukemia was ruled out by peripheral blood and bone marrow aspirate smears, and all sorts of pathogen and tumor associated antigen were negative. Intravascular T cell lymphoma was confirmed by biopsy and immunohistochemical studies, followed by combination chemotherapy yielding complete response, and now was being followed up. Conclusions IVL is an unusual form of non Hodgkin's lymphoma characterized by intravascular proliferation of atypical lymphoid cells in multiple organs, with a predilection for skin and nervous system involvement. Lymph node involvement is quite rare, especially IVL diagnosed by biopsy of the superficial lymph node has not been reported; Mainly B cell immunophenotypes and rare T cell are found; Diagnosis is dependent on biopsy, often favourable responses to chemotherapy ,but prognosis is generally poor. The pathogenesis remains unknown.