Abstract

This case study explores a clinicopathological presentation of Adult T-cell leukaemia/lymphoma (ATLL) at Tygerberg Hospital; a disease associated with adulthood noted in an adolescent patient. Adult T-cell leukaemia–lymphoma oncogenesis develops through a multistep process with an accumulation of mutations. Infection through human T-lymphotropic virus type 1 (HTLV-1) is the first step of a multistep process resulting in eventual clonal proliferation of mature T-cells. There is a long latency period of 20–50 years from the time of infection with HTLV-1 to the development of symptoms of ATLL; thus, ATLL is a malignancy associated with adulthood. The median age of diagnosis is 58, ranging from the third to ninth decade of life. This is an ideal learning case as it highlights the importance of recognising ATLL in children and young adults in our population.

Highlights

  • Human T-lymphotropic virus type 1 (HTLV-1) is a retrovirus that infects different types of cells including T cells, B cells, fibroblasts, dendritic cells and macrophages

  • Transmission of HTLV-1 by blood transfusion is more commonly associated with myelopathy or tropical spastic paraparesis, while vertical transmission is more commonly associated with the development of Adult T-cell leukaemia/lymphoma (ATLL).[1]

  • We present a case of an adolescent with ATLL

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Summary

Introduction

Human T-lymphotropic virus type 1 (HTLV-1) is a retrovirus that infects different types of cells including T cells, B cells, fibroblasts, dendritic cells and macrophages. Human T-lymphotropic virus type 1 infects regulatory T cells that express CD4, CD25 and FOXP3. Transmission of HTLV-1 by blood transfusion is more commonly associated with myelopathy or tropical spastic paraparesis, while vertical transmission is more commonly associated with the development of Adult T-cell leukaemia/lymphoma (ATLL).[1]. Human T-lymphotropic virus type 1 infection is the first event in this multistep process which results in clonal CD4+ T cell proliferation.[1,7] There is a long latency period of 20–50 years from the time of HTLV-1 infection to the development of symptoms of ATLL.[1] Given the long latency period, ATLL as the name says is a disease occurring in adulthood with a median age at diagnosis of 58 years and it is extremely rare in childhood.[1,8]. Positron emission tomography–computed tomography scan 6 months later showed complete remission

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