细胞淋巴瘤
进行性多灶性白质脑病
医学
活检
病理
脑脊液
淋巴瘤
皮肤活检
白质脑病
脑活检
放射科
多发性硬化
疾病
免疫学
作者
Elisabetta Belli,Chiara Milano,Ilaria Pesaresi,Ilaria Trivelli,Antonio Tavoni,Eugenio Ciancia,Greta Alì,Virna Zampa,Chiara Pizzanelli,Gabriele Siciliano,Giulia Ricci
标识
DOI:10.1080/00207454.2021.1972418
摘要
Intravascular large B-cell lymphoma (IVLBCL) is a very rare form of extranodal lymphoma, characterized by the proliferation of neoplastic B cells within the lumen of small vessels. Due to its high aggressivity, for years the prognosis had been really poor with only anectodical cases of remission after traditional chemotherapy. More recently, new therapeutic protocols allowed a significant increase in overall survival. It can virtually involve every organ, being skin and central nervous system the most affected. The clinical presentation is often unspecific and insidious; therefore, diagnosis can be challenging. Tissue biopsy, in particular random deep skin biopsy, is the gold standard for definitive diagnosis. We describe the case of a 58-year-old woman with a previous diagnosis of myelofibrosis, who presented with a rapidly progressive neurological deterioration and a brain MRI suggestive of Progressive Multifocal Leukoencephalopathy. Due to the absence of BK and JC viruses in cerebrospinal fluid and the presence of severe myalgias and subcutaneous nodules, a skin and muscle biopsy was performed, allowing diagnosis of IVLBCL. We describe the diagnostic pitfalls of this case, briefly reviewing existing literature about IVLBCL.
科研通智能强力驱动
Strongly Powered by AbleSci AI