初発時にCMVとHHV-6の再活性化と頭蓋内多発腫瘤を認めた成人T細胞白血病リンパ腫

症例は55歳，男性。1ヶ月前からの咽頭痛の精査目的で紹介された。意識障害と項部硬直，口腔内の多発性有痛性潰瘍，全身に米粒大の紅斑を認めた。白血球数7,910/μL (異常リンパ球2%)，LDH 203U/L，補正カルシウム11.2mg/dL,可溶性IL-2受容体11,800U/mL，サイトメガロウイルス抗原(C10，C11)43/49。末梢血や骨髄，皮膚にCD4，25陽性の異常リンパ球あり，末梢血でHTLV-1プロウイルスDNAのモノクローナルな組み込みを確認し，成人T細胞白血病リンパ腫(ATLL)と診断した。CTで，脳実質内にリング状造影効果を有する腫瘤が多発し，髄液検査で細胞数1,320/mm3 (フローサイトメトリー法でATLL細胞が79%)，蛋白244mg/dL，HHV-6 DNA陽性を認めた。ヘルペスウイルス属感染症に対する治療や髄注を併用してmodified LSG15療法を行うも，意識障害が急速に進行し，入院21日目で死亡した。ATLLでは，中枢神経浸潤のさらなる病態の解明と標準治療の確立が求められる。

A 55-year-old man, suffering from a sore throat for a month was referred to our hospital. Physical examination revealed disturbance of consciousness, nuchal rigidity, painful multiple ulcers in the oral cavity and rice grain sized erythema on the whole body. Hematological examination showed the following: white blood cell 7,910/μL (abnormal lymphocytes 2%), LDH 203U/L, corrected calcium 11.2mg/dL, soluble IL-2 receptor 11,800U/mL, and cytomegalovirus antigenemia (C10,C11) 43/49. Abnormal lymphocytes (CD4+CD25+) were found in the peripheral blood, bone marrow and skin samples. Southern blotting of peripheral blood confirmed monoclonal integration of HTLV-1 provirus DNA, consequently, he was diagnosed with adult T-cell leukemia/lymphoma (ATLL). Contrast-enhanced computed tomography revealed multiple tumors with ringed contrast effect in the brain parenchyma. Examination of the cerebrospinal fluid showed that the cell number was 1,320/mm3 (ATLL cells were 79% in flow cytometry), and the protein level was 244mg/dL; further, the examination revealed a positive result for human herpesvirus 6 DNA. Although treatment for herpesvirus genus and modified LSG15 therapy combined with intrathecal chemotherapy were performed, the patient became comatose, and he died on day 21 of hospitalization. A deeper understanding of the pathogenesis of and the establishment of standard treatment for ATLL with CNS involvement are required.