血管内淋巴瘤

Intravascular lymphoma of Iymphod: a case report and Iiterature review

目的 探讨血管内淋巴瘤 (intravascularlymphoma ,IVL)的临床及病理特征 ,提高对其认识。 方法 1例伴神经系统表现的淋巴结IVL的临床、病理观察分析并复习文献。结果 男性 ,3 1岁 ,不明原因高热伴双下肢乏力、消瘦 5 0天 ,右腹股沟直径 1cm淋巴结 1枚 ,双下肢肌力Ⅱ～Ⅲ级 ,B超示肝脏轻度增大 ,血LDH明显升高 ( 10 70U )伴ESR及转氨酶轻度升高 ,外周血WBC 3 .3× 10 9/L ,骨髓像、多种病原及各肿瘤抗原检测均无异常。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LCA、UCHL -1、CD3 阳性 ,CK、CD68、CD79α、CD2 0 、CD3 1、CD3 4 均阴性。行CHOP化疗后症状缓解 ,现仍在随访中。结论 IVL是一罕见非何杰金淋巴瘤 ,好发于中枢神经系统及皮肤 ,其他部位少见 ,B细胞型多见 ,T细胞型罕见 ,临床表现有一定提示性 ,确诊靠组织学 ,对化疗敏感 ,预后差。

Objective To investigate the clinical and pathological features of IVL. Methods A case of IVL of inguinal lymph node was studied with clinical findings, histopathology and immunohistochemisty as well as literature review. Results A case of 31-year-old man presented with unknown fever and inertia of both low extremities and weight loss for 50 days, one of the right inguinal lymph node was found, muscle power of both low extremities was Ⅱ—Ⅲ grade, liver was light enlargement, and with elevation of serum LDH、GOP、GTP and ESR, leukemia was ruled out by peripheral blood and bone marrow aspirate smears, 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 this patient is being followed-up. Conclusion IVL is an unusual form of non-Hodgkin lymphoma characterized by intravascular proliferation of atypical lymphoid cells in multiple organs, with a predilection for nervous system and skin involvement, Lymphod involvement is quite rare, Mainly B-cell immunophenotypes and rare T-cell, diagnosis is dependent on biopsy, often favourable responses to chemotherapy, but prognosis is generally poor, the pathogenesis remains unknown.