鼻副鼻腔より眼窩及び頭蓋内に連続する腫瘤を形成した成人T細胞白血病の1例

Intracranial and Orbital Extension of a Nasal Cavity Adult T-Cell Leukemia : a case report

I．はじめに

　ATLとはHTLV-I感染により引き起こされるT細胞の白血病・リンパ腫である．ATLはさまざまな発症形態をとり，臨床症状も複雑であり，診断が困難なことがある．今回われわれは臨床的に進行性鼻疽lethal midline granulomaとして発症し眼窩，頭蓋内に進展したATLの症例を経験したので報告する．

Adult T-cell leukemia (ATL) is one kind of leukemia induced by human T lymphotropic virus type I(HTLV-I) infection. An unusual case of ATL is presented. A fifty-one-year-old male patient was admittedto our hospital because of nasal obstruction and blindness in the left eye. Imaging study revealed a masslesion in the nasal cavity, the left paranasal sinus extending to the left orbit and intracranial frontal base.Biopsy of the mass from the paranasal sinus was carried out and the histological diagnosis was a granulo-matous lesion with non-specific inflammation. The clinical impression of the lesion was lethal midline gra-nuloma. After steroid therapy and 50Gy of local radiotherapy, the patient's symptoms disappeared exceptfor his blindness in the left eye. Imaging study revealed that the mass lesion had become smaller. In spiteof local improvement, new lesions such as cervical lymph node swelling and multiple nodular shadows inthe lung fields appeared on CT scan. Histological diagnosis of the biopsied cervical lymph node was T-celldominant non-Hodgkin's lymphoma of the diffuse type. Serologically, anti-HTLV-I antibody was positive.Southern blot analysis of lymph node biopsy showed monoclonal proliferation of ATL cells. We made thediagnosis of our case as ATL. The patient died 16 months later despite repeated systemic chemotherapywith cyclophosphamide, vincristine, adriamycin, and prednisolone. ATL can involve the central nervoussystem (CNS) and manifest CNS symptoms. The neurosurgeon also should consider the CNS involvementof ATL especially in Japan.