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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 intravascular lymphoma (IVL) and to improve its understanding. Methods One case of lymph node with neurological manifestations of IVL clinical and pathological analysis and review of the literature. Results Male, 31 years old, unexplained fever with bilateral lower extremity fatigue, weight loss 50 days, 1 cm in diameter of the right groin lymph nodes, muscle strength of both lower extremities Ⅱ ~ Ⅲ grade, B ultrasound showed a slight increase in liver, blood LDH was significantly increased High (1070U) with mild ESR and transaminase, peripheral blood WBC 3 .3 × 10 9 / L, bone marrow imaging, a variety of pathogens and the detection of tumor antigens no abnormalities. Pathological biopsy: most of inguinal lymph node destruction, replaced by a large number of small and medium-sized expansion of the blood vessels, the cavity is filled with a large number of atypical lymphoid cells, with associated wall, peritubular infiltration and involvement of extranodal adipose tissue. The tumor cell immunophenotype LCA, UCHL -1, CD3 positive, CK, CD68, CD79α, CD20, CD3 1, CD3 4 were negative. CHOP chemotherapy after the line to relieve symptoms, is still under follow-up. Conclusions IVL is a rare non-Hodgkin’s lymphoma, which occurs in the central nervous system and skin. It is uncommon in other parts of the body. B cell type is rare, T cell type is rare, and clinical manifestations are somewhat suggestive. Sensitive, poor prognosis.