Tomasini Carlo, Novelli Mauro, Ponti Renata, Pippione Mario, Bernengo Maria Grazia
Department of Biomedical Science and Human Oncology, Second Dermatologic Clinic, University of Turin, Turin, Italy.
Dermatology. 2004;208(2):158-63. doi: 10.1159/000076493.
Intravascular lymphoma (IL) is a rare aggressive disease characterized by exclusive or predominant accumulation of malignant lymphoid cells within the lumina of small arteries, veins and capillaries. We describe a 74-year-old woman with IL presenting in the skin as asymptomatic teleangiectatic plaques on the thighs. The cutaneous neoplasm developed 18 months after an extravascular large B cell lymphoma of the lung and heralded its relapse. No neurologic symptoms were present during the course of the fatal disease. Immunophenotypic and immunogenotypic studies confirmed a unique B cell subtype. This case of IL is unusual for the association with an extravascular B cell lymphoma suggesting a link between the two diseases.
血管内淋巴瘤(IL)是一种罕见的侵袭性疾病,其特征是恶性淋巴细胞在小动脉、静脉和毛细血管腔内排他性或主要聚集。我们描述了一名74岁患有IL的女性,其皮肤表现为大腿上无症状的毛细血管扩张性斑块。皮肤肿瘤在肺部血管外大B细胞淋巴瘤发生18个月后出现,并预示着其复发。在这种致命疾病的病程中未出现神经症状。免疫表型和免疫基因型研究证实为一种独特的B细胞亚型。该例IL与血管外B细胞淋巴瘤相关,这种情况不常见,提示这两种疾病之间存在联系。