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What’s adult T-cell leukemia?

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Adult T-cell leukemia is a rare and aggressive cancer caused by HTLV-1, a retrovirus that can be transmitted through blood, sexual contact, or mother to child. Symptoms include skin lesions, enlarged lymph nodes, and susceptibility to infections. Diagnosis is made by biopsy and treatment involves chemotherapy, antiviral drugs, and bone marrow transplantation.

Adult T-cell leukemia (ATL) is a rare type of cancer that develops in immune T cells. ATL is caused by human T-cell leukemia/lymphoma virus type 1, or HTLV-1. This cancer is aggressive and most patients die within a year of diagnosis. Adult T-cell leukemia is a form of non-Hodgkin lymphoma. ATL affects adults and is most common in the Caribbean and Japan. It is seen in less frequent clusters in Western countries.

HTLV-1, the virus responsible for ATL is a retrovirus. It can be transmitted through blood, such as shared needles or a blood transfusion, sexual contact, or from mother to child. The latency period for the development of adult T-cell leukemia after exposure to HTLV-1 averages 30 years. Fewer than five percent of people who carry HTLV-1 develop ATL.

The visible symptoms of adult T-cell leukemia include skin lesions and enlarged lymph nodes. Other symptoms include increased susceptibility to infections, high blood calcium levels, bone lesions, and enlarged liver and spleen. There are four subtypes of the disease, acute and lymphomatous, both of which grow very rapidly, and the less aggressive smoldering and chronic ATL.

Symptoms develop rapidly in acute ATL. The patient typically has swollen lymph nodes in the groin, armpit and neck, fatigue, skin rashes, and high blood calcium levels, which can lead to severe constipation and an irregular heartbeat. Lymphomatous ATL is confined to lymph nodes. Chronic ATL progresses slowly, and symptoms include enlarged lymph nodes and enlarged organs such as the spleen and liver, as well as skin rashes and fatigue. Smoking ATL progresses very slowly and has mild symptoms such as lesions on the skin.

The diagnosis of adult T-cell leukemia is made by biopsy. The healthcare professional will look at the abnormal skin tissue or tumor tissue through a microscope to make a diagnosis. He or she may also do a blood test to measure blood calcium levels, as well as white blood cell counts. Once the diagnosis is made, the doctor may order a CT scan of the spleen, liver, chest and abdomen, as well as a bone marrow biopsy to determine the extent of the disease.

Because adult T-cell leukemia is relatively rare, it is difficult to conduct clinical trials to develop a recommended treatment protocol. The standard treatment for ATL is similar to other forms of T-cell lymphoma, which is a combination of chemotherapy drugs. Patients can also take antiviral drugs and interferon to treat HTLV-1 infection. Some healthcare professionals recommend bone marrow transplantation for patients going into remission.

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