Su Derrick W, Pasch Whitney, Costales Cristina, Siddiqi Imran, Mohrbacher Ann
Jane Anne Nohl Division of Hematology (Su, Mohrbacher) and the Hematopathology Section, Department of Pathology (Pasch, Costales), Norris Comprehensive Cancer Center and Hospital, University of Southern California, Los Angeles, California.
Proc (Bayl Univ Med Cent). 2017 Apr;30(2):186-189. doi: 10.1080/08998280.2017.11929579.
Intravascular large B-cell lymphoma (IVLBCL) is a rare and deadly malignancy involving the growth of lymphoma cells within vessel lumina of all organ types. IVLBCL is further divided into the hemophagocytic Asian variant and a classical Western variant. Both variants are difficult to diagnose by imaging, and although diagnostic criteria have been developed to guide workup, histopathological examination remains imperative. Treatment of IVLBCL remains difficult given the high mortality of the disease, but rituximab has emerged as a promising therapeutic option when combined with various cytotoxic regimens. The two main variants of IVLBCL generally manifest in their respective Asian or Western populations, and crossover between ethnicities is rare. We present the second described case of Asian-variant IVLBCL in an African American individual.
血管内大B细胞淋巴瘤(IVLBCL)是一种罕见的致命性恶性肿瘤,其特征是淋巴瘤细胞在所有器官类型的血管腔内生长。IVLBCL进一步分为噬血细胞性亚洲变异型和经典西方变异型。两种变异型均难以通过影像学诊断,尽管已制定诊断标准以指导检查,但组织病理学检查仍然至关重要。鉴于该疾病的高死亡率,IVLBCL的治疗仍然困难,但利妥昔单抗与各种细胞毒性方案联合使用时已成为一种有前景的治疗选择。IVLBCL的两种主要变异型通常分别在其亚洲或西方人群中表现,不同种族之间的交叉情况很少见。我们报告了第二例非裔美国人中亚洲变异型IVLBCL的病例。