Ocular Manifestations and Pathology of Adult T-cell Leukemia/Lymphoma Associated with Human T-lymphotropic virus Type 1

Abstract

The human T-cell lymphotropic virus type 1 (HTLV-1), endemic in defined geographical areas around the world, is recognized as the etiologic agent of adult T-cell leukemia/lymphoma (ATL), or HTLV-1. ATL is a rare adult onset T-cell malignancy that is characterized by the presence of ATL flower cells with T-cell markers, HTLV-1 antibodies in the serum, and monoclonal integration of HTLV-1 provirus in affected cells. Ocular manifestations associated with HTLV-1 virus infection have been reported and include HTLV-1 uveitis and keratoconjunctivitis sicca, but reports of ocular involvement in ATL are exceedingly rare. This article describes the ocular manifestations and pathology of ATL. We also report for the first time a case of a 34-year-old male with systemic ATL and prominent atypical lymphoid cell infiltration in the choroid. To our knowledge, this is the first report defining prominent choroidal involvement as a distinct ocular manifestation of ATL. ATL may masquerade as a variety of other conditions, and molecular techniques involving microdissection and PCR have proven to be critical diagnostic tools. International collaboration will be needed to better understand the presentation and diagnosis of this rare malignancy.

Magnesium Level in Patients With Adult T-Cell Leukemia: A Case Report

“…ATL is characterized by systemic lymphadenopathy and/or extranodal lesions, leukemic changes in the blood, and positive retroviral serology for HTLV-1 (8). Most common sites of the extranodal ATL involvement are skin, liver, spleen, bone and bone marrow (9).…”

A new era of uveitis: impact of polymerase chain reaction in intraocular inflammatory diseases

“…Numerous case reports demonstrate that ATL cells can infiltrate almost all ocular tissues, such as the orbit, cornea, iris, lens, vitreous, uvea, retina, sclera, and optic nerve [92]. Kamoi et al recently reported a distinctive characteristic of ATL cell infiltration into the eye [93]: the ATL cells prefer to form segmental multiple rounded nodules, especially at the palpebral conjunctiva around the lacrimal punctum during their infiltration into the ocular mucous membrane (Fig.…”

Ocular and Adnexal Lymphoma

“…HTLV-1 infection is endemic in the Caribbean Islands, Japan, South America, and Central Africa, with transmission through direct contact with bodily fl uids (Kumar et al 1994 ;Liu et al 2010 ). HTLV-1 infection is endemic in the Caribbean Islands, Japan, South America, and Central Africa, with transmission through direct contact with bodily fl uids (Kumar et al 1994 ;Liu et al 2010 ).…”

“…A proportion of 1-3 % of HTLV-1-infected individuals develop ATLL after prolonged viral persistence, usually two decades after the infection (Grassmann et al 2005 ). 10.1 ) Levy-Clarke et al 2002 ;Liu et al 2010 ). Tax protein is the primary HTLV-1-encoded factor used by the virus to stimulate T-cellular proliferation.…”