Kong Yun-Yi, Dai Bo, Sheng Wei-Qi, Yang Wen-Tao, Wang Chao-Fu, Kong Jin-Cheng, Shi Da-Ren
Department of Pathology, Cancer Hospital, Shanghai, China.
J Cutan Pathol. 2009 Aug;36(8):865-70. doi: 10.1111/j.1600-0560.2008.01168.x. Epub 2008 Nov 24.
Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare type of malignant lymphoma characterized by exclusive or predominant growth of neoplastic cells within the lumen of blood vessels. Cases in the literature predominantly involve the skin and central nervous system, with special emphasis on the 'cutaneous variant'.
Three cases of IVLBCL with cutaneous manifestations, including two systemic IVLBCL and one cutaneous variant, were described in this study. In all cases, clinical presentation and follow-up data were meticulously evaluated and immunophenotypic and molecular studies were performed.
All three cases displayed the B-cell phenotype and showed monoclonality with immunoglobulin heavy chain gene rearrangement. Bcl2 was expressed in the two systemic IVLBCL cases with fatal outcomes. The third patient with the 'cutaneous variant' achieved complete remission and a longer survival time of 15 months after chemotherapy.
Skin manifestations and neurological findings, although to different degrees, are important clues to the diagnosis of IVLBCL. As most IVLBCL are grouped into the post-germinal center B-cell subtype of diffuse large B-cell lymphoma, Bcl2 expression may be correlated with a worse prognosis in IVLBCL. The cutaneous variant of IVLBCL has a significantly better outcome than that of systemic IVLBCL.
血管内大B细胞淋巴瘤（IVLBCL）是一种极其罕见的恶性淋巴瘤，其特征为肿瘤细胞在血管腔内排他性或占主导地位地生长。文献中的病例主要累及皮肤和中枢神经系统，特别强调“皮肤变异型”。
本研究描述了3例有皮肤表现的IVLBCL病例，包括2例系统性IVLBCL和1例皮肤变异型。对所有病例的临床表现和随访数据进行了细致评估，并进行了免疫表型和分子研究。
所有3例均表现为B细胞表型，免疫球蛋白重链基因重排显示单克隆性。在2例结局致命的系统性IVLBCL病例中检测到Bcl2表达。第3例“皮肤变异型”患者化疗后达到完全缓解，生存期延长至15个月。
皮肤表现和神经系统表现，尽管程度不同，但都是诊断IVLBCL的重要线索。由于大多数IVLBCL归类于弥漫性大B细胞淋巴瘤的生发中心后B细胞亚型，Bcl2表达可能与IVLBCL预后较差相关。IVLBCL的皮肤变异型预后明显优于系统性IVLBCL。
