ADULT T-CELL LEUKEMIA/LYMPHOMA

By Gary M. White, MD


Adult T-cell leukemia/lymphoma (ATL) is a peripheral T-lymphocytic neoplasm associated with infection by human T-cell lymphotrophic virus type-1 (HTLV-1).

Clinical

A variety of presentations may occur including large papulosquamous plaques or red scaly areas indistinguishable from CTCL, disseminated papules, papulonodules or tumors, and an erythroderma similar to Sézary syndrome. This disease is associated with infection by HTLV-1. PCR has detected HTLV-1 in saliva which may represent another potential source for transmission. People from south-west Japan, the Caribbean basin, West and Central Africa, and South and Central America are most commonly affected. Sporadic cases occur in the US, Middle East, Africa, and Latin America. Hypercalcemia and osteolytic bone lesions may occur. Some patients develop a cellular immune deficiency which allows opportunistic infections to develop. The disease has been categorized into 4 different types:

The acute and lymphomatous types have median survival rates of less than a year.

Diagnosis

Any patient with a histologically or cytologically proven peripheral T-cell malignancy who resides in an endemic area should have serology for HTLV-1 performed.

Treatment

Referral to a specialist is appropriate. Multiple newer agents are being investigated. The monoclonal antibody to CCR4, mogamulizumab, has improved response rates in patients with treatment-naïve and relapsed aggressive ATL.

References

A rare presentation of adult T-cell leukemia/lymphoma with generalized cutaneous purpuric lesions Dermatologica Sinica 33 (2015) 234--238

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