脳生検術により確定診断され早期に化学療法を開始された血管内リンパ腫の1例

Intravascular Lymphoma Confirmed by Brain Biopsy,Quickly Treated by Chemotherapy: A Case Report

Ⅰ．はじめに

　血管内リンパ腫intravascular lymphoma（IVL）は，中枢神経や皮膚をはじめとして全身諸臓器の小血管内で腫瘍細胞が増殖する稀な疾患である．本疾患に特異的な症状や画像・検査所見に乏しく，症状の進行も急速で予後不良であることから，生前の確定診断は困難と言われている．今回われわれは，開頭生検術により確定診断し早期より化学療法を開始することができた1例を経験したので，若干の文献的考察を加えて報告する．

　A 60-year-old female with intravascular lymphoma (IVL) presented with the complaint of urinary dysfunction and gait disturbance. T2 weighted MR imaging of the thoracic spinal cord showed a hyperintense lesion, and brain MR imaging indicated hyperintense lesions in the deep white matter. Multiple sclerosis was assumed, so steroid pulse therapy was administered. However, her level of consciousness decreased and her paraplegia progressed. Laboratory data showed that anemia and thrombocytopenia had worsened with high serum LDH and soluble IL-2 receptor levels. Biopsy of bone marrow indicated hypercellularity associated with hemophagocytic histiocytes, although no atypical lymphocytes were detected. Brain MR imaging indicated a new subcortical lesion in the left parietal lobe. One and a half months after admission, an open brain biopsy of the left parietal cortex was performed. Histopathological diagnosis was IVL, large B cell type. Immediately, she underwent CHOP therapy containing rituximab (R-CHOP therapy). After chemotherapy, spinal and brain MR images showed no new abnormal lesions.

　Clinically, it is difficult to make a diagnosis of IVL in life as it has no characteristic symptoms or radiological findings. Therefore, if a patient is suspected of having IVL, a biopsy of different organs, including brain, is necessary for making an early diagnosis and initiating chemotherapy.