RESUMEN
Background: Adult T-cell leukemia/lymphoma (ATL), one of the most aggressive cancers in the world, occurs in 5% of the 10 million people living with HTLV-1 worldwide. French Guiana, a French overseas territory in South America, is one of the highest endemic areas of HTLV-1 worldwide. Here, we describe the demographic and clinical characteristics and outcome of ATL in this area. Methods: We retrospectively collected data from all patients diagnosed between 2009 and 2019. Patients were distributed according to Shimoyama's classification. Prognostic factors were explored through univariate analysis. Findings: Over the 10-year study period, 41 patients with a median age of 54 years at diagnosis were identified, among whom 56% were women. Sixteen (39%) patients were Maroons, a cultural group descendant of the runaway enslaved Africans from former Dutch Guiana. Among the study population, 23 (56%) had an acute type, 14 (34%) a lymphoma type, and one and one chronic and primary cutaneous tumour, respectively. First-lines of treatment included either chemotherapy or Zidovudine combined with pegylated interferon alpha. The 4-year overall survival was 11.4% for the entire population with 0% and 11% for lymphoma and acute forms, respectively. The median progression-free survival was 93 and 115 days for the acute and lymphoma groups (p = 0.37), respectively. Among the twenty-nine patients who died, 8 (28%) died of toxicity, 7 (24%) died of disease progression and the cause of death remained unknown in 14 (48%) patients. Due to the overall poor prognosis, no significant prognostic factors could be identified. Interpretation: This study provides real-life data from ATL patients in French Guiana, a remote territory in a middle-income region. Patients, mostly Maroons, presented with a younger age and the prognosis was worse than expected compared to Japanese patients. Funding: None.
