Nakahara T, Saito T, Muroi A, Sugiura Y, Ogata M, Sugiyama Y, Yamamoto T
Department of Neurology, School of Medicine, Fukushima Medical University, Japan.
J Neurol Neurosurg Psychiatry. 1999 Sep;67(3):403-6. doi: 10.1136/jnnp.67.3.403.
A 63 year old man developed dysaesthesia in the legs followed by a subacute ascending flaccid paraparesis with sacral sensory and autonomic involvement. Intravascular lymphomatosis (IVL) was favoured by the presence of low grade fever and raised serum C reactive protein, CSF pleocytosis, raised lymphoma markers (serum LDH, soluble IL-2 receptor), and steroid responsiveness. Only muscle, among several organ biopsies, confirmed IVL. A cytogenetic study of the bone marrow showed chromosome 6 monosomy, as previously reported. The monosomy of chromosome 19, which bears the intercellular cell adhesion molecule-1, newly found in this case, may be related to the unique tumour embolisation of IVL. The CHOP regimen (six courses in 12 weeks) using granulocyte colony stimulating factor (G-CSF) led to gradual resolution of myeloradiculopathy and laboratory supported remission lasting for more than 13 months. The biweekly CHOP with G-CSF support may be a choice of chemotherapy in averting rapidly fatal IVL.
一名63岁男性出现腿部感觉异常,随后出现亚急性上升性弛缓性截瘫,并伴有骶部感觉和自主神经受累。低热、血清C反应蛋白升高、脑脊液细胞增多、淋巴瘤标志物升高(血清乳酸脱氢酶、可溶性白细胞介素-2受体)以及对类固醇有反应,这些表现提示血管内淋巴瘤(IVL)。在多处器官活检中,仅肌肉活检确诊为IVL。骨髓细胞遗传学研究显示有6号染色体单体,如先前报道。本病例新发现携带细胞间黏附分子-1的19号染色体单体,可能与IVL独特的肿瘤栓塞有关。使用粒细胞集落刺激因子(G-CSF)的CHOP方案(12周内6个疗程)使脊髓神经根病逐渐缓解,实验室检查支持缓解持续超过13个月。每两周一次的CHOP方案加G-CSF支持可能是避免IVL迅速致命的化疗选择。