Nagasaki Akitoshi, Miyagi Takashi, Taira Tamiko, Shinhama Akihiko, Kojya Shizuo, Suzuki Mikio, Aonahata Miyuki, Yoshimi Naoki, Takasu Nobuyuki
Department of Endocrinology and Metabolism, Internal Medicine, University of the Ryukyus School of Medicine, Okinawa, Japan.
Head Neck. 2008 Jun;30(6):815-20. doi: 10.1002/hed.20730.
Adult T-cell leukemia/lymphoma (ATLL) is a highly aggressive T-cell lymphoma and etiologically associated with human T-lymphotropic virus type 1 (HTLV-1). Patients with ATLL commonly present with leukemic changes, systemic lymphadenopathy, and/or extranodal lesion and have very poor prognosis.
We describe a rare case of ATLL presenting as an isolated paranasal mass. Southern blot analysis of the biopsied specimens demonstrated multiple integration bands of HTLV-1 provirus of different intensities. Chemotherapy resulted in complete resolution of the paranasal mass. Thereafter, the patient showed an indolent clinical course with leukemic changes and pulmonary and cutaneous ATLL lesions and remains alive more than 5 years from diagnosis.
ATLL should be included in the differential diagnosis of sinonasal lymphoma, although the event is rare. Multiple HTLV-1 provirus integrations of different intensities may be indicative of good prognosis for ATLL.
成人T细胞白血病/淋巴瘤（ATLL）是一种侵袭性很强的T细胞淋巴瘤，在病因上与人嗜T淋巴细胞病毒1型（HTLV-1）相关。ATLL患者通常表现为白血病改变、全身淋巴结肿大和/或结外病变，预后很差。
我们描述了一例罕见的以孤立性鼻旁肿块为表现的ATLL病例。对活检标本进行Southern印迹分析显示HTLV-1前病毒有多个强度不同的整合带。化疗使鼻旁肿块完全消退。此后，患者临床病程呈惰性，出现白血病改变以及肺部和皮肤ATLL病变，自诊断后存活超过5年。
尽管这种情况罕见，但ATLL应列入鼻窦淋巴瘤的鉴别诊断中。不同强度的多个HTLV-1前病毒整合可能提示ATLL预后良好。
