皮肤血管内大B细胞性淋巴瘤临床病理观察  

Intravascular large B-cell lymphoma with skin: a clinicopathologic analysis and review of literature

作　　者：王晓开[1] 宋福林[1] 秦海明[1] 匡丽[1] 

机构地区：[1]沈阳军区总医院病理科,沈阳110016

出　　处：《诊断病理学杂志》2015年第3期142-145,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨皮肤血管内大B细胞性淋巴瘤(B-IVL)的临床病理特征。方法通过光镜及免疫组化检测,分析1例B-IVL的临床病理特征及免疫组化特点。结果患者女性,67岁。临床表现为发热伴双侧大腿内侧红斑,后形成硬块,不痛不痒,双下肢浮肿。血化验显示全血细胞进行性降低;骨髓穿刺未见异常。皮肤活检镜下见皮下脂肪层毛细血管内有较大的肿瘤性淋巴细胞。免疫组化示肿瘤细胞LCA、CD3(背景细胞)、CD20和C-myc(+),bcl-2少数(+),bcl-6散在(+),mum-1少部分(+);CD10和CD5(-)。Ki-67阳性指数>30%,EBER原位杂交(-),淋巴瘤基因重排检测结果发现有单克隆性增生的B细胞亚群。确诊后采用R-CHOP方案化疗1个疗程,体温恢复正常。结论 B-IVL为一种较为罕见的非霍奇金恶性淋巴瘤,最常累及皮肤和中枢神经系统,可累及肾、肺、肾上腺、胃肠道和软组织等全身多个脏器,约30%病例累及皮肤,好发于下肢,病变多局限于血管内;病理检查及免疫组化、基因重排对确定诊断有重要意义。本病预后差,联合化疗有助于提高疗效。Objective To report a case of intravascular large B-cell lymphoma（ B-IVL） of skin and to study the clinicopathologic features of IVL with review of the literature. Methods A case of B-IVL of skin was studied by light microscopy and immunohistochemistry staining for LCA,CD3,CD20,C-myc,bcl-2,bcl-6,mum-1,CD10,CD5,Ki-67.Results The patient,a 67-year-old Chinese woman,had hypertension,diabetes and fever with bilateral thigh erythema,accompanied by lumps after exacerbations,superficial,lower extremity edema. Tests showed reduced whole blood cells,bone marrow biopsy showed no abnormalities. Skin biopsy showed that larger lymphocytoid tumor cells were noted within capillaries in subcutaneous fat layer. Immunohistochemistry showed that the tumor cells were LCA（ ＋）,CD3（ background cell ＋）,CD20（ ＋）,C-myc（ ＋）,bcl-2（ ＋）,bcl-6（ ＋）,mum-1（ ＋）,CD10（-）,CD5（-） and Ki-67（ ＋） 30%. EBER in situ hybridization was negative,and Ig gene rearrangement analysis found that there was monoclonal proliferation of B cell subsets. After diagnosis a course of R-CHOP regimen was used,and the body temperature returned to normal, with significant efficacy. Conclusion B-IVL as a relatively rare malignant non-Hodgkin ＇ s lymphoma,most commonly involves the skin and CNS,but it may also involve the kidney, lung, adrenal gland,gastrointestinal tract and soft tissue. About 30% of cases involve the skin in the lower extremities,lesions are confined to the blood vessels. Pathology,immunohistochemistry and gene rearrangement analysis are important for the diagnosis. The prognosis is poor. Combined chemotherapy contributes to disease treatment.

关 键 词：血管内淋巴瘤 B细胞 皮肤 免疫组化 

分 类 号：R732.2[医药卫生—肿瘤]