Adult T-cell leukemia/lymphoma (ATLL)

Definition

Adult T-cell leukemia/lymphoma (ATLL) is a type of cancer that affects the T-cells, specifically the CD4+ T-lymphocytes, and is associated with infection by the Human T-lymphotropic virus type I (HTLV-I). This malignancy often manifests in adults, leading to a variety of symptoms such as skin lesions, lymphadenopathy, and immune dysfunction. The connection to HTLV-I highlights the role of oncogenic viruses in the development of certain cancers, illustrating how viral infections can have long-term impacts on human health.

5 Must Know Facts For Your Next Test

1. ATLL is primarily found in regions where HTLV-I is endemic, such as parts of Japan, the Caribbean, and sub-Saharan Africa.
2. The disease has an aggressive clinical course, with various subtypes classified based on clinical presentation, including acute, chronic, and smoldering forms.
3. Symptoms can include hypercalcemia, skin lesions, and bone marrow infiltration leading to cytopenias.
4. Diagnosis typically involves blood tests showing increased lymphocyte counts, along with genetic testing for HTLV-I proviral DNA.
5. Treatment options may include chemotherapy, antiviral therapy targeting HTLV-I, and stem cell transplantation for eligible patients.

Review Questions

• How does HTLV-I infection contribute to the development of Adult T-cell leukemia/lymphoma?
  • HTLV-I infection leads to the transformation of CD4+ T-lymphocytes into malignant cells. The virus integrates its genetic material into the host's DNA, disrupting normal cellular regulation and promoting uncontrolled cell proliferation. This oncogenic process contributes to the development of ATLL, showcasing the direct link between viral infections and cancer formation.
• Discuss the clinical manifestations of Adult T-cell leukemia/lymphoma and how they differ among its subtypes.
  • Adult T-cell leukemia/lymphoma can present with diverse symptoms depending on its subtype. Acute ATLL often leads to rapid onset of lymphadenopathy, skin lesions, and hypercalcemia. In contrast, chronic ATLL may exhibit milder symptoms with persistent lymphocyte counts but can progress to acute phases. The smoldering form displays asymptomatic characteristics yet may eventually evolve into more aggressive manifestations.
• Evaluate the treatment strategies for Adult T-cell leukemia/lymphoma and their effectiveness in managing this disease.
  • Treatment for Adult T-cell leukemia/lymphoma generally includes chemotherapy regimens aimed at reducing tumor burden and managing symptoms. In cases where chemotherapy fails or recurs, options like stem cell transplantation or antiviral therapies targeting HTLV-I may be considered. However, ATLL often shows resistance to conventional therapies due to its aggressive nature and the underlying viral infection, making early diagnosis and intervention crucial for improving patient outcomes.

Related terms

Human T-lymphotropic virus type I (HTLV-I): HTLV-I is a retrovirus that infects T-cells and is associated with various diseases, including ATLL and tropical spastic paraparesis.

Oncogenic viruses: Oncogenic viruses are viruses that can cause cancer in humans by promoting uncontrolled cell division or inhibiting apoptosis.

Lymphoma: Lymphoma is a type of cancer that originates in lymphocytes, which are part of the immune system, and can be either Hodgkin or non-Hodgkin lymphoma.