TY - JOUR
T1 - Updates in lymph node and skin pathology of adult T-cell leukemia/lymphoma, biomarkers, and beyond
AU - Adkins, Brian D.
AU - Ramos, Juan C.
AU - Bliss-Moreau, Meghan
AU - Gru, Alejandro A.
N1 - Publisher Copyright:
© 2019 Elsevier Inc.
PY - 2020/1
Y1 - 2020/1
N2 - Adult T-cell leukemia/lymphoma (ATLL) is a mature T-cell lymphoproliferative disorder associated with the human T lymphotropic virus (HTLV-1) infection. ATLL predominantly affects individuals within HTLV-1 endemic areas such as Japan, areas of Africa, South America, and the Caribbean. HTLV-1 preferentially infects CD4+ T-cells, and several genetic hits must occur before ATLL develops. ATLL is classically divided into four clinical variants based on manifestations of disease: acute, chronic, lymphomatous, and smouldering. As of 2019, a new subtype has been described: lymphoma type of ATL, extranodal primary cutaneous. In this review, emphasis will be taken to describe the common clinicopathologic manifestations of the disease, advances in biomarker discovery, mutational landscape and targeted therapeutic approaches to treat this highly aggressive and frequently lethal type of T-cell lymphoma.
AB - Adult T-cell leukemia/lymphoma (ATLL) is a mature T-cell lymphoproliferative disorder associated with the human T lymphotropic virus (HTLV-1) infection. ATLL predominantly affects individuals within HTLV-1 endemic areas such as Japan, areas of Africa, South America, and the Caribbean. HTLV-1 preferentially infects CD4+ T-cells, and several genetic hits must occur before ATLL develops. ATLL is classically divided into four clinical variants based on manifestations of disease: acute, chronic, lymphomatous, and smouldering. As of 2019, a new subtype has been described: lymphoma type of ATL, extranodal primary cutaneous. In this review, emphasis will be taken to describe the common clinicopathologic manifestations of the disease, advances in biomarker discovery, mutational landscape and targeted therapeutic approaches to treat this highly aggressive and frequently lethal type of T-cell lymphoma.
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U2 - 10.1053/j.semdp.2019.12.006
DO - 10.1053/j.semdp.2019.12.006
M3 - Review article
C2 - 31889601
AN - SCOPUS:85076980265
SN - 0740-2570
VL - 37
SP - 1
EP - 10
JO - Seminars in Diagnostic Pathology
JF - Seminars in Diagnostic Pathology
IS - 1
ER -