Adult T-cell leukemia/lymphoma with multiple integration of HTLV-1 provirus presenting as an isolated paranasal sinus tumor: a case report.
BACKGROUND
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.


RESULTS
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.


CONCLUSIONS
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.
Nagasaki
Akitoshi
A
Department of Endocrinology and Metabolism, Internal Medicine, University of the Ryukyus School of Medicine, Okinawa, Japan. anaga@ryudai2nai.com
Miyagi
Takashi
T
Taira
Tamiko
T
Shinhama
Akihiko
A
Kojya
Shizuo
S
Suzuki
Mikio
M
Aonahata
Miyuki
M
Yoshimi
Naoki
N
Takasu
Nobuyuki
N
D000328:Adult; D015368:Human T-lymphotropic virus 1 / Q000502:physiology*; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000175:diagnosis* / Q000628:therapy / Q000821:virology; D008297:Male; D010255:Paranasal Sinus Neoplasms / Q000175:diagnosis* / Q000628:therapy / Q000821:virology; D011533:Proviruses / Q000502:physiology*; D016662:Virus Integration