Adult T-cell leukemia/lymphoma
Alternate names[edit | edit source]
ATLL; Adult T-cell leukaemia; Adult T-cell leukaemia/lymphoma (HTLV-1 positive); Adult T-cell lymphoma
Definition[edit | edit source]
(uh-DULT T-sel loo-KEE-mee-uh/lim-FOH-muh)An aggressive (fast-growing) type of T-cell non-Hodgkin lymphoma caused by the human T-cell leukemia virus type 1 (HTLV-1). It is marked by bone and skin lesions, high calcium levels, and enlarged lymph nodes, spleen, and liver. Also called ATLL.
Epidemiology[edit | edit source]
- ATLL and HTLV‐I are prevalent in Japan, the Caribbean basin, and certain regions of South America and Africa, and in immigrants from these countries to other regions. [1].
- It has also been recognised in patients originating from Iran and Central Europe.
- In the UK, ATLL is mainly, but not exclusively, seen in first or second generation immigrants of Afro‐Caribbean descent.
Cause[edit | edit source]
- It is aetiologically linked to the human T‐cell lymphotropic virus, HTLV‐I.
- HTLV‐I is detected in cells from virtually all cases of ATLL, with exceedingly rare exceptions.
Types[edit | edit source]
There are four basic clinical variants of ATL: acute (60% of cases), lymphomatous (20 % of cases), chronic (10% of cases) and smoldering (10% of cases).
Signs and symptoms[edit | edit source]
- The clinical features of ATL include generalized swelling of the lymph nodes (lymphadenopathy), increased liver and spleen size (hepatosplenomegaly), immunosuppression, high levels of calcium in the blood, lytic bone lesions (spots that appear as “holes” on a standard bone x-ray), and skin lesions.
Diagnosis[edit | edit source]
- The diagnosis should be based on a constellation of clinical features and laboratory investigations. [2].
- The latter comprise: lymphocyte morphology, immunophenotype, histology of the tissues affected in the pure lymphoma forms and serology or DNA analysis for HTLV‐I.
Treatment[edit | edit source]
- The best treatment for these patients is unclear and patients should be enrolled in clinical trials whenever possible.
- Medication may include CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) or EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide and prednisone).
- In some patients, a bone marrow transplant may be recommended.[3].
References[edit | edit source]
- ↑ Matutes E. (2007). Adult T-cell leukaemia/lymphoma. Journal of clinical pathology, 60(12), 1373–1377. https://doi.org/10.1136/jcp.2007.052456
- ↑ Matutes E. (2007). Adult T-cell leukaemia/lymphoma. Journal of clinical pathology, 60(12), 1373–1377. https://doi.org/10.1136/jcp.2007.052456
- ↑ Matutes E. (2007). Adult T-cell leukaemia/lymphoma. Journal of clinical pathology, 60(12), 1373–1377. https://doi.org/10.1136/jcp.2007.052456
NIH genetic and rare disease info[edit source]
Adult T-cell leukemia/lymphoma is a rare disease.
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