Adult T-cell leukemia/lymphoma

A type of cancer affecting T-cells

Adult T-cell leukemia/lymphoma (ATLL) is a rare and aggressive type of cancer that affects the T-cells of the immune system. It is associated with infection by the Human T-lymphotropic virus type 1 (HTLV-1). ATLL is characterized by the proliferation of malignant T-cells and can present in various clinical forms.

Classification

ATLL is classified into four main clinical subtypes:

• Acute ATLL: This is the most aggressive form, characterized by rapid progression and a high number of circulating malignant T-cells.
• Lymphoma-type ATLL: This form presents primarily with lymphadenopathy and does not have a significant leukemic component.
• Chronic ATLL: This subtype has a slower progression and is often associated with skin lesions and mild lymphadenopathy.
• Smoldering ATLL: This is the least aggressive form, with minimal symptoms and a slow progression.

Pathophysiology

ATLL is caused by the HTLV-1 infection, which leads to the transformation of T-cells into malignant cells. The virus integrates into the host genome and affects cellular pathways that control cell proliferation and apoptosis. The exact mechanism of transformation is complex and involves multiple genetic and epigenetic changes.

Epidemiology

ATLL is most prevalent in regions where HTLV-1 is endemic, such as southwestern Japan, the Caribbean, parts of Central Africa, and South America. The disease primarily affects adults, with a higher incidence in individuals over the age of 40.

Clinical Features

The clinical presentation of ATLL varies depending on the subtype. Common symptoms include:

• Lymphadenopathy
• Hepatosplenomegaly
• Skin lesions
• Hypercalcemia
• Fatigue
• Fever

Diagnosis

Diagnosis of ATLL involves a combination of clinical evaluation, laboratory tests, and imaging studies. Key diagnostic criteria include:

• Detection of HTLV-1 antibodies in the blood
• Abnormal T-cell morphology in peripheral blood smears
• Immunophenotyping to identify T-cell markers
• Genetic testing for HTLV-1 integration

Treatment

Treatment options for ATLL depend on the subtype and severity of the disease. Common approaches include:

• Chemotherapy
• Antiviral therapy
• Stem cell transplantation
• Monoclonal antibody therapy

Prognosis

The prognosis for ATLL varies by subtype, with acute and lymphoma-type ATLL having a poorer prognosis compared to chronic and smoldering forms. Overall, the disease is associated with a high mortality rate, and long-term survival is limited.

Prevention

Preventive measures focus on reducing the transmission of HTLV-1, including:

• Screening blood donors for HTLV-1
• Promoting safe sex practices
• Educating at-risk populations

Related pages

• Human T-lymphotropic virus 1
• Leukemia
• Lymphoma
• T-cell

Gallery

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  A healthy human T-cell

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