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There are four subtypes of adult T-cell leukemia. Do you know which one is the most deadly?
Adult T-cell leukemia/lymphoma (ATL) is a rare cancer caused by human T-cell leukemia/lymphotropic virus type 1 (HTLV-1) that primarily affects the T cells of the immune system. The majority of ATL cells harbor integrated HTLV-1 provirus, further confirming the pathogenic role of this virus in tumorigenesis. Among people infected with HTLV-1, only a small percentage will develop ATL, and there is usually a long incubation period between infection and the development of ATL.
Four types of ATL
Based on pathological characteristics, adult T-cell leukemia is divided into four subtypes: acute, latent, lymphoma and chronic. The acute and lymphoma types are known for their highly aggressive nature and poor prognosis. A clear understanding of the characteristics of these subtypes and their impact on patient outcomes is critical to driving targeted treatment options.
ATL typically develops at around age 62, but the median age of diagnosis varies depending on the prevalence of HTLV-1 in different regions.
Symptoms and Signs
ATL often presents as a highly aggressive non-Hodgkin lymphoma that often lacks distinctive histologic features. Circulating lymphocytes with irregular nuclei are frequently observed and are considered leukemic cells. Typical symptoms in infected individuals include visceral involvement, hypercalcemia, skin lesions, and osteolytic bone lesions. Compared with other hematological malignancies, patients with HTLV-1-induced ATL are more prone to tumor-induced osteolysis and hypercalcemia.
Most ATL patients die within a year of diagnosis, demonstrating the cruelty and lethality of this disease.
Transmission channels
HTLV-1 is mainly transmitted from mother to child, through sexual contact and contaminated blood. Infection may also occur if blood is transferred or contaminated needles are shared.
Diagnostic Methods
The diagnosis of ATL relies on a combination of clinical features, morphological and immunophenotypic changes characteristic of malignant cells. According to the Shimoyama classification, ATL is divided into acute, lymphoma, chronic and latent types, allowing clinicians to provide the most suitable treatment options for different subtypes.
Confirmation of the presence of HTLV-1 and identification of at least 5% of tumor cells in peripheral blood is usually sufficient to diagnose acute, chronic, and latent forms.
Treatment options
Current treatment options for ATL are generally based on clinical subtype and response to initial therapy. Treatment options include various chemotherapy regimens, acyclovir and interferon combination therapy, and autologous hematopoietic stem cell transplantation. Recently, a selective treatment for relapsed or refractory ATL has been approved in Japan.
EpidemiologyIn 2021, antibody therapeutics for relapsed or refractory ATL also began to appear in clinical practice.
In the United States, HTLV-1 infection rates are low; although substantial serologic data are lacking, rates are presumed to be highest among African Americans in the Southeast. The overall prevalence of HTLV-1 is also increasing, while ATL is relatively rare. From Haiti to Africa to Japan, the virus has spread to many parts of the world.
Research Progress
New approaches to the treatment of PTCL are being actively investigated. New compounds are gaining attention, with increasing emphasis on their expected efficacy in the relapsed or refractory setting.
In the face of this deadly disease, how can we better improve our understanding of adult T-cell leukemia so that it can be detected early and effectively treated?
