Adult T-Cell Leukemia/Lymphoma-Related Ocular Manifestations: Analysis of the First Large-Scale Nationwide Survey

Abstract

Adult T-cell leukemia/lymphoma (ATL) is a rare and aggressive T-cell malignancy with a high mortality rate, resulting in a lack of information among ophthalmologists. Here, we investigated the state of ophthalmic medical care for ATL and ATL-related ocular manifestations by conducting the first large-scale nationwide survey in Japan. A total of 115 facilities were surveyed, including all university hospitals in Japan that were members of the Japanese Ophthalmological Society and regional core facilities that were members of the Japanese Ocular Inflammation Society. The collected nationwide data on the state of medical care for ATL-related ocular manifestations and ATL-associated ocular findings were categorized, tallied, and analyzed. Of the 115 facilities, 69 (60%) responded. Overall, 28 facilities (43.0%) had experience in providing ophthalmic care to ATL patients. ATL-related ocular manifestations were most commonly diagnosed “based on blood tests and characteristic ophthalmic findings.” By analyzing the 48 reported cases of ATL-related ocular manifestations, common ATL-related ocular lesions were intraocular infiltration (22 cases, 45.8%) and opportunistic infections (19 cases, 39.6%). All cases of opportunistic infection were cytomegalovirus retinitis. Dry eye (3 cases, 6.3%), scleritis (2 cases, 4.2%), uveitis (1 case, 2.1%), and anemic retinopathy (1 case, 2.1%) were also seen. In conclusion, intraocular infiltration and cytomegalovirus retinitis are common among ATL patients, and ophthalmologists should keep these findings in mind in their practice.

HTLV-1 in Ophthalmology

“…Analyzing such reports, ocular infiltration of ATL was the most frequent manifestation, observed in almost all tissues in and around the eye, and with reports mainly arising from Japan (Goto et al, 1993;Shibata et al, 1997;Mori et al, 2003;Yoshikawa et al, 2005;Miyamura et al, 2009;Liu et al, 2010;Maruyama et al, 2013;Hirano et al, 2017;Shimono et al, 2017;Sabundayo et al, 2018;Yamada et al, 2019), followed by Martinique (Merle et al, 2005(Merle et al, , 2016Fares et al, 2018) and the United States (Liu et al, 2010;Larson et al, 2012). In 2018, our nationwide survey in Japan confirmed that the most frequent ATL-related ocular manifestation was ATL infiltration (Kamoi et al, 2018).…”

“…However, all manifestations have been reported as case reports or case series because of the rarity of cases and the difficulty of documentation resulting from the severe condition and high fatality rate of patients. In the late 2010s, we performed a nationwide survey and identified the frequency of ATL-related ocular manifestations (Kamoi et al, 2018).…”

“…Compared with other forms of leukemia, ATL cells are much more adept at infiltrating the eyes (Kamoi et al, 2018). Previous case reports have indicated that ATL cells have the potential to infiltrate into various tissues in the eye such as the orbit (Mori et al, 2003;Fares et al, 2018), conjunctiva (Takahashi et al, 1993), lacrimal glands (Sabundayo et al, 2018), cornea (Buggage et al, 2001;Miyamura et al, 2009), vitreous humor (Ohba et al, 1989;Kohno et al, 1993;Hirata et al, 2002;Maruyama et al, 2013), uvea (Mori et al, 2003), retina (Ohba et al, 1989;Kohno et al, 1993;Hirata et al, 2002), choroid (Liu et al, 2010), and optic nerve (Yamamoto et al, 1994).…”

“…When considering the mechanisms underlying this characteristic infiltration, analysis of the vitreous humor appears informative. Our nationwide survey (Kamoi et al, 2018) identified intraocular infiltration and opportunistic infections as common ATL-related ocular lesions. All cases of opportunistic infection involved cytomegalovirus retinitis.…”

The challenge of describing the epidemiology of HTLV in the Amazon region of Brazil

“…HTLV integrates the transcribed RNA as a DNA provirus into the cell nucleic acid [12][13][14][15][16], and this simple evolutionary procedure leads to the persistence of the virus and its maintenance in nature and has serious implications for the different clinical and epidemiological outcomes of the infection and diseases associated with the virus. The wide array of clinical outcomes shows the target complexity within the human host (including the CNS, blood, lungs, eyes, muscles, bladder and skin), and several medical specialties have to be involved in the care and treatment of infected and diseased persons [17][18][19][20][21][22][23][24][25][26][27].…”