Adult T-cell lymphomaDescription, Causes and Risk Factors:An acute or subacute disease associated with a human T-cell virus, with lymphadenopathy, hepatosplenomegaly, skin lesions, peripheral blood involvement, and hypercalcemia.Adult T-cell lymphoma (ATL) is a rare and often aggressive (fast-growing) T-cell lymphoma that can be found in the blood (leukemia), lymph nodes (lymphoma), skin, or multiple areas of the body. ATL has been linked to infection by the human T-cell lymphotropic virus type 1 (HTLV-1); however, less than 5% of individuals with the HTLV-1 will develop ATL.There are four subtypes of Adult T-cell lymphoma (ATL): acute, lymphomatous, chronic, and smoldering. Acute and lymphomatous are fast-growing forms of ATL, whereas chronic and smoldering are less aggressive:Acute: In individuals with acute ATL, symptoms develop rapidly and may include fatigue, skin rash, and enlarged lymph nodes in the neck, armpit, or groin. The hallmarks of acute ATL are a high white blood cell count often accompanied by an elevated level of calcium in the blood (hypercalcemia), which can cause irregular heart rhythms and severe constipation.
Lymphomatous: This subtype of ATL is found primarily in the lymph nodes.
Chronic: This slow-growing type of ATL can result in elevated lymphocytes in the blood, enlarged lymph nodes, skin rash, or fatigue. It can also be found in other areas of the body such as the spleen and liver.
Smoldering: This slow-growing type of ATL is associated with very mild symptoms, such as a few skin lesions.
Currently, physicians have no way of predicting which infected patients will develop Adult T-cell lymphoma (ATL). The HTLV-1 virus is most common in southern Japan and the Caribbean. In Europe and North America, the virus is found chie?y in immigrants from the endemic areas and in some communities of intravenous drug users.HTLV-I is transmitted via three major routes: (i) transmission from mother-to-child by breast feeding; (ii) transmission through sexual contact; (iii) transmission by infected blood, either by blood transfusion or by the contaminated needles among drug abusers.Symptoms of Adult T-cell lymphoma:Muscle weakness.
Spasticity in the lower extremities.
Urinary disturbance associated with preferential damage of the thoracic spinal cord.
Diagnosis:Depending on the subtype, diagnosing Adult T-cell lymphoma may require removing a small sample of tumor tissue or abnormal skin tissue, called a biopsy, and looking at the cells under a microscope. A blood test may also be necessary to measure the white blood cell count and calcium levels. Other tests, such as a bone marrow biopsy, a computed axial tomography (CAT) scan of the chest, abdomen, liver, and spleen, and/or a positron emission tomography (PET) scan may be used to determine if, or how far, the cancer has spread.Treatment:Observation without treatment may be appropriate for patients with mild or no symptoms, although follow-up monitoring is required. For ATL affecting the skin, skin-directed therapies (for example, topical steroids or local radiation) may be prescribed.Because ATL is such a rare disease, there have not been enough patients enrolled in Clinical trials to establish treatment standards in the United States & Europe, especially for the acute and lymphomatous subtypes. As a result, common frontline therapies used to treat ATL are the same as those used to treat other types of T-cell lymphomas. These include:CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone).
EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone).
Other treatments may include zidovudine (RetrovirTM), also known as AZTTM, in combination with interferon-alpha to treat the underlying HTLV-1 virus infection. This may be effective in people with the slower growing forms of the disease. In some patients, a bone marrow transplant may be appropriate following remission.Similar to the frontline setting, standard treatment for relapsed ATL has not been established. Many regimens used to treat other T-cell lymphomas following relapse are also being used to treat ATL, including the following. These include:GND (gemcitabine, vinorelbine, and doxorubicin).
ICE (ifosfamide, carboplatin, and etoposide).
DHAP (dexamethasone, cytarabine, and cisplatin).
CEPP (cyclophosphamide, etoposide, procarbazine, and prednisone).
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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