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Adult T-cell leukemia/lymphoma

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Alternate names

ATLL; Adult T-cell leukaemia; Adult T-cell leukaemia/lymphoma (HTLV-1 positive); Adult T-cell lymphoma

Definition

(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

  • 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

  • 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

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

  • 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

  • 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


References

  1. Matutes E. (2007). Adult T-cell leukaemia/lymphoma. Journal of clinical pathology, 60(12), 1373–1377. https://doi.org/10.1136/jcp.2007.052456
  2. Matutes E. (2007). Adult T-cell leukaemia/lymphoma. Journal of clinical pathology, 60(12), 1373–1377. https://doi.org/10.1136/jcp.2007.052456
  3. Matutes E. (2007). Adult T-cell leukaemia/lymphoma. Journal of clinical pathology, 60(12), 1373–1377. https://doi.org/10.1136/jcp.2007.052456

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NIH genetic and rare disease info

Adult T-cell leukemia/lymphoma is a rare disease.


Latest research - Adult T-cell leukemia/lymphoma

PubMed
Clinical trials



Latest research - Adult T-cell leukemia/lymphoma

PubMed
Clinical trials


 

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