We report one of the largest non-Japanese cohorts of adult T-cell leukemia/lymphoma (ATL). A total of 175 cases were identified in Martinique between 1983 and 2013. The sex ratio was 1.01. The median age was 56 years. The overall incidence rate was 1.16/105 inhabitants/year, with a risk 1.29 times higher for men. The distribution of clinical types (acute, lymphoma, and chronic) was 62.9%, 29.1%, and 8%, respectively. Median survival time was 3.06, 8.13, and 45.16 months, respectively, for the acute, lymphoma, and chronic types (p <0.001). Survival was significantly higher for lymphoma type with skin lesions (median 13.96 months versus 6.06, p <0.002) and for the acute type without hypercalcemia (5.1 versus 2.4 months, p <0.01). Symptoms associated with hypercalcemia present in 46.9% of patients and skin lesions in 43.4% were the best-performing clinical signs for the diagnosis of ATL. However, only 16.9% had these manifestations. Strongyloides stercoralis infection was documented in 42.5% of patients. Twenty-three patients had an atypical phenotype, including 14 cases with CD4-CD8- and 4 with CD4+CD8+. Twenty-four patients did not express CD25 with no significant impact on overall survival. The hyperploidy, trisomy of chromosome 3 and rearrangements of chromosome 14 were the most frequent karyotype abnormalities.
Keywords: HTLV-1, ATL, epidemiology of ATL, phenotype of ATL cell, karyotype of ATL cell.
A Case of Adult T-cell Leukemia-Lymphoma (Lymphoma Type) of Waldeyer’s Ring