側頸部から上位頸椎へ進展した成人前駆型Bリンパ芽球性リンパ腫の1例

An Adult Case of Precursor B Cell Lymphoblastic Lymphoma Extending from Right Neck to Upper Cervical Spinal Region

Ⅰ．はじめに

　リンパ芽球性リンパ腫（lymphoblastic lymphoma，LBL）は悪性リンパ腫のなかでも最も悪性度が高く，non-Hodgkin lymphoma（NHL）の2～4％を占める^1,10)．急性リンパ性白血病に類似し^2,4,5,8)，LBLの大多数がT細胞性で，小児期や思春期の男性に発症しやすい^6,8,11)．好発部位は，縦隔や横隔膜より上のリンパ節であり^6,8)，初診時に中枢神経系に病変が及ぶものは9％程度で¹²⁾，脊椎腫瘍を認めるものは稀である．今回われわれは，右側頸部から上部頸椎硬膜外に進展した稀な前駆型Bリンパ芽球性リンパ腫（B cell lymphoblastic lymphoma，BLBL）の成人例を経験したので報告し，診断方法と治療について考察した．

We report the rare adult case of upper cervical spinal tumor diagnosed precursor B cell lymphoblastic lymphoma. A 48- year-old male had suffered from right neck pain and swelling for two months. He had no neurological symptoms. The serum level of IL-2 receptor was high (1,820 U/ml). The radiological examinations including MRI showed the tumor extending from right neck to the epidural space from medulla to the C4 level. The pathological diagnosis of biopsy specimens was malignant lymphoma. Since the early pre-B lymphoblast antigens were positive by the flow cytometry,the diagnosis was precursor B cell lymphoblastic lymphoma. This type of lymphoma is highly aggressive. The intensive chemotherapy regimen such as hyper-CVAD was superior to the lymphoma-like regimens. In the case showed the progressive neurological symptoms such as myelopathy and urinary incontinence,the immediate surgical decompression of the spinal cord may be necessary. Measurement of IL-2 receptor and biopsy with flow cytometry were necessary to work out the treatment strategy of the spinal malignant lymphoma. In this case,the complete response (CR) of the tumor was achieved with hyper-CVAD regimen and radiation therapy.