血管内大B细胞淋巴瘤

A case of intravascular large B cell lymphoma

报告1例血管内大B细胞淋巴瘤。患者男,48岁。因面、颈部红斑2年,发热、气促及结膜充血3个月就诊。皮肤科检查:双眼睑水肿;双颊部对称性淡红斑伴毛细血管扩张,眶周、鼻唇沟无受累;颈部、颈前"V"字区弥漫性片状红斑,境界清楚,压之缓慢褪色,无浸润感;双上肢散在不规则淡红斑,双手指间关节处隐约有淡红斑,无浸润感。皮损组织病理检查:真皮及皮下组织血管腔内见异形淋巴样细胞。免疫组化:CD20、CD45、B细胞淋巴瘤因子(Bcl)-2、Bcl-6、CD79a、配对盒子基因(PAX)5及多发性骨髓瘤癌基因(MUM)1均(+),增殖核抗原(Ki-67)(90%+),CD5(弱+),C-myc(约20%弱+),CD3、细胞角蛋白(CK)、CD10、D型细胞周期蛋白1(Cyclin D1)、末端脱氧核苷酸转移酶(TdT)、半乳糖凝集素(Gal)-3、髓过氧化物酶(MPO)及前列腺特异性抗原(PSA)均(-)。诊断:血管内大B细胞淋巴瘤。

A case of intravascular large B cell lymphoma is reported.A 48-year-old male patient presented with erythema on the face and neck for 2 years,and fever,wheeziness,and conjunctival congestion for 3 months.Physical examination showed symmetrical light erythema and telangiectasia on cheeks,but no erythema was observed around the eyes or in the nasolabial folds.Erythema with clear boundary can be seen on the neck and anterior region of neck,fading slowly without infiltration when pressed.Irregular light erythema was scattered on both upper arms,and light red plaques were faintly visible at the interpha-langeal joints of both hands,without infiltration when pressed.Histopathological examination of erythema in anterior region of neck showed atypia lymphoid cells in the vascular lumen of dermis and the subcutaneous tissue.Immunohistochemical exami nation showed that tumor cells in the vascular lumen were positive for CD20,CD45,B-cell lymphoma(Bel)-2,Bcl-6,CD79a,paired box gene(PAX)5 and multiple myeloma oncogene(MUM)1,90%positive for cell proliferation-associated nuclear antigen(Ki-67),about 20%weakly positive for C-myc,weakly positive for CD5,but negative for CD3,cytokeratin(CK),CD 10,D-type cyclin proteins 1(Cyclin D1),terminal deoxynucleotidyl transferase(TdT),galectin(Gal)-3,myeloperoxidase(MPO)and prostate specific antigen(PSA).The diagnosis intravascular large B cell lymphoma was made.