adult T-cell leukemia/lymphoma

Adult T-cell leukemia/lymphoma (ATLL) is caused by the HTLV-1 virus, endemic to Japan and the Caribbean, and is likely derived from cells with the T-regulatory phenotype.

The malignant cells express IL2 receptor α (CD25), and the majority express transcription factor Forkhead box P3 (Foxp3), in addition to T-cell markers. Occasional cases express CD30.

Whereas Japanese cases are predominantly of the acute and chronic leukemic types, the less well-studied Caribbean cases are more often lymphomatous.

Foxp3 expression correlates with pleomorphic small and medium cell types and may be lost with large cell transformation.

The evolution of the latter type can be associated with the gain of CD30 expression; such ATLL tumors might respond to anti-CD30 monoclonal antibody therapies.

References

 Loss of Foxp3 Is Associated With CD30 Expression in the Anaplastic Large Cell Subtype of Adult T-cell Leukemia/Lymphoma (ATLL) in US/Caribbean Patients: Potential Therapeutic Implications for CD30 Antibody-mediated Therapy. Yao J, Gottesman SR, Ayalew G, Braverman AS, Axiotis CA. Am J Surg Pathol. 2013 Jun 20. PMID: 23797717

 Proietti FA, Carneiro-Proietti AB, Catalan-Soares BC, Murphy EL. Global epidemiology of HTLV-I infection and associated diseases. Oncogene. 2005 Sep 5;24(39):6058-68. PMID: 16155612

 Taylor GP, Matsuoka M. Natural history of adult T-cell leukemia/lymphoma and approaches to therapy. Oncogene. 2005 Sep 5;24(39):6047-57. PMID: 16155611