Adult t cell leukemia

The disease manifests with leukaemia in greater than two thirds of patients, while the remaining patients have a lymphomatous form. According to the disease manifestations, various forms which differ in clinical course and prognosis have been recognised: acute, chronic, smouldering and lymphoma. The diagnosis should be based on a constellation of clinical features and laboratory investigations. The disease is recognised under the WHO classification as a distinct entity. It has also been recognised in patients originating from Iran and Central Europe. Usually the disease is disseminated, although lymphomas confined to the gut and central nervous system have been recognised.
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Adult T‐cell leukaemia/lymphoma

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Adult T‐cell leukaemia/lymphoma

ATL is a type of T-cell lymphoma — a lymphoma that develops from white blood cells called T cells. Low-grade types of ATL can sometimes change transform into faster-growing types. ATL is very rare. Only around 30 people are diagnosed with it in the UK each year.
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Adult T-cell leukemia/lymphoma

HTLV-1 is a human retrovirus that is endemic in certain parts of the world, including the northern and southern islands of Japan, the Caribbean, parts of Sub-Saharan Africa, parts of South America, and the Middle East. The most common routes of transmission are mother to child through breast feeding, blood transfusions, or sexual transmission. Human T-cell lymphotrophic virus type 2 HTLV-2 was thought to carry an increased risk; however currently this paradigm is now questioned as to whether it causes any known human disease. There are several distinct clinical presentations termed: smoldering, chronic, acute, and lymphoma.
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ATL is usually a highly aggressive non-Hodgkin's lymphoma with no characteristic histologic appearance except for a diffuse pattern and a mature T-cell phenotype. ATL is frequently accompanied by visceral involvement, hypercalcemia , skin lesions , and lytic bone lesions. Bone invasion and osteolysis , features of bone metastases, commonly occur in the setting of advanced solid tumors, such as breast, prostate, and lung cancers, but are less common in hematologic malignancies. However, patients with HTLV-1—induced ATL and multiple myeloma are predisposed to the development of tumor-induced osteolysis and hypercalcemia. One of the striking features of ATL and multiple myeloma induced bone disease is that the bone lesions are predominantly osteolytic with little associated osteoblastic activity.
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