Epidemiology of Adult T-Cell Leukaemia/Lymphoma in South Africa over a 10-Year Period

Abstract

Introduction. Adult T-cell leukaemia/lymphoma (ATLL) is a rare and aggressive malignancy of mature T-cells. Limited epidemiological studies have shown that there is substantial variation in age at diagnosis and subtype distribution between different geographical regions. This is the first epidemiological study of ATLL in South Africa. Methods. A national epidemiological study of ATLL in South Africa was performed. All new cases of ATLL from 2009 to 2019 were identified by laboratory database search in public and private health care sectors. Demographic and diagnostic data were obtained, and the cases were subtyped according to the Shimoyama classification. Results. There were 31 patients with ATLL over the 10-year period, with an incidence of 0.06 per 100000 population. The male to female ratio was 1 : 1 and the median age at diagnosis was 37 years. Acute ATLL was the most commonly seen subtype in South Africa. Conclusion. In this, the first epidemiological study of ATLL in South Africa, we demonstrate that ATLL is a rare disease, that acute ATLL is the most commonly diagnosed subtype, and that ATLL is likely under diagnosed. Patients present at a considerably younger age than the reported age in other nations.

Geographic distribution, clinical epidemiology and genetic diversity of the human oncogenic retrovirus HTLV-1 in Africa, the world’s largest endemic area

“…In Mali, rare cases of ATL have been diagnosed at the Institut Marchoux in Bamako (133,134), together with other cutaneous T-cell lymphomas, such as Seźary syndrome (133). Surprisingly, only 36 cases of ATL have been diagnosed in South Africa despite the high level of endemicity of HTLV-1, and the presence of a hospital system and virology research facilities that are much developed than those in many other sub-Saharan African countries (191)(192)(193)(194)(195).…”