Adult T-cell leukemia/lymphoma

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Adult T-cell leukemia/lymphoma
Healthy Human T Cell
Synonyms N/A
Pronounce N/A
Specialty N/A
Symptoms Lymphadenopathy, hepatosplenomegaly, skin lesions, hypercalcemia
Complications Infection, organ failure
Onset Typically in adulthood
Duration Chronic
Types Acute, Lymphomatous, Chronic, Smoldering
Causes Human T-lymphotropic virus 1 (HTLV-1)
Risks HTLV-1 infection, immunosuppression
Diagnosis Blood test, biopsy, imaging studies
Differential diagnosis Non-Hodgkin lymphoma, chronic lymphocytic leukemia
Prevention N/A
Treatment Chemotherapy, antiviral therapy, stem cell transplant
Medication N/A
Prognosis Variable, often poor
Frequency Rare
Deaths N/A


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[edit | edit source]

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[edit | edit source]

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[edit | edit source]

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[edit | edit source]

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

Diagnosis[edit | edit source]

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[edit | edit source]

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

Prognosis[edit | edit source]

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[edit | edit source]

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[edit | edit source]

Gallery[edit | edit source]

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Contributors: Prab R. Tumpati, MD