ABSTRACT
BACKGROUND: To report a case of lymphomatous infiltration and bilateral retinal vasculitis observed among 83 cases of adult T-cell leukemia (ATL) treated in the University Hospital Center in Fort-de-France (Martinique, French West Indies) between 1984 and 2003. CASE: A complete clinical ophthalmologic examination was performed in this patient along with fluorescein angiography. OBSERVATIONS: After being checked for diffuse adenopathies, myodesopsias, and phosphenes, the 35-year-old patient was diagnosed with ATL. The ocular impairment, present since the onset of ATL as peripheral subretinal infiltrates, spread progressively and afferently to the rest of the retina in the form of an essentially venous vasculitis. Impairment of the vitreous was noted only in the end stages of disease progression. As ocular lesions progressed, the general state of the patient degraded at the same time despite chemotherapeutic measures. CONCLUSION: Among the more than 300 seropositive for human T-cell lymphotropic virus type 1 (HTLV-1) or patients with HTLV-1-associated myelopathy/tropical spastic paraparesis treated at our hospital in the last 20 years, and among the 83 cases of ATL, only this single case of retinal vasculitis associated with HTLV-1 was observed (1/83, 1.2%) in Martinique, confirming the geographic variability of the clinical phenotype of HTLV-1 infection. The incidence of retinal vasculitis in ATL patients may signify an even worse prognosis than initially indicated.
Subject(s)
Human T-lymphotropic virus 1/isolation & purification , Leukemia-Lymphoma, Adult T-Cell/complications , Retinal Vasculitis/etiology , Adult , Antiviral Agents/therapeutic use , Blotting, Western , Drug Therapy, Combination , Enzyme-Linked Immunosorbent Assay , Fatal Outcome , Female , Fluorescein Angiography , HTLV-I Antibodies/blood , Human T-lymphotropic virus 1/immunology , Humans , Leukemia-Lymphoma, Adult T-Cell/diagnosis , Leukemia-Lymphoma, Adult T-Cell/drug therapy , Leukemic Infiltration , Retina/pathology , Retinal Vasculitis/diagnosis , Retinal Vasculitis/drug therapyABSTRACT
UNLABELLED: Adult T-cell leukemia-lymphoma (ATLL) has a very bad prognosis and remains resistant to conventional therapy. Promising results have been reported with the combination of zidovudine (AZT) and alpha-interferon (IFN). METHOD: A combination with IFN and antinucleoside [AZT or zalcitabine (ddC)] was applied since 1995 in Martinique (French West Indies). An initial treatment with two cycles of CHOP was added to reduce initial tumoral burden, followed by antiretroviral (ARV) therapy associated with etoposide. We report the characteristics and outcomes of 29 patients diagnosed with an ATLL between 1990 and 1999. The overall median survival was 8 months. A striking improvement of survival was observed when comparing the periods between 1990-1994 and 1995-1999 (17 months versus 3 months, p = 0.004). During the second period, seven patients received a therapy with oral etoposide, antinucleoside and IFN, among which, six patients received an initial induction CHOP chemotherapy. No major toxicity was observed with this strategy. In conclusion, the progression of survival since 1995 suggests that a therapeutic approach combining initial polychemotherapy with CHOP followed by ARV drugs, IFN and oral etoposide is an interesting option in treating patients with ATLL.
