The importance of the clinical classification of adult T-cell leukemia/lymphoma (ATLL) in the prognosis

• Published in: PLoS neglected tropical diseases 2022-10, Vol.16 (10), p.e0010807
• Main Authors: Pedro Dantas Oliveira, Guilherme Sousa Ribeiro, Rosangela Oliveira Anjos, Maria Almeida Dias, Lourdes Farre, Iguaracyra Araújo, Achiléa Lisboa Bittencourt
• Format: Article
• Language: English
• ISSN: 1935-2727; 1935-2735
• DOI: 10.1371/journal.pntd.0010807

BackgroundAdult T-cell leukemia/lymphoma (ATLL), a peripheral T-cell leukemia/lymphoma associated with the human T-cell lymphotropic virus type-1 (HTLV-1), has been classified following the clinical forms defined by Shimoyama in 1991. A suggestion to modify Shimoyama's classification was proposed in 2007 to differentiate within the smoldering patients those who presented nodules or tumors in the skin without lung involvement, which was named the primary cutaneous tumoral (PCT) form of ATLL. In the present study, according to their clinicopathological characteristics, we estimated the mortality rates of 143 ATLL patients from Bahia, Brazil. We also evaluated the importance of classifying PCT/ATLL separately from the smoldering type on disease prognosis.Methodology/principal findingsDiagnosis of ATLL was established based on a positive serology for HTLV-1, histopathological and/or cytological diagnosis of peripheral T-cell leukemia/lymphoma. Patients were clinically grouped according to Shimoyama's classification, considering PCT variants separately from the smoldering cases. Bivariate and multivariable survival analyses were applied to identify factors associated with disease prognosis. Significant differences in the median survival time were observed between the clinical types, with the smoldering type presenting the longest median survival (109 months) compared to the other forms (