Overview
Adult T cell leukemia-lymphoma (ATLL) is a rare and aggressive lymphoid cancer that only occurs in people living with HTLV-1 infection. The exact mechanism that leads to ATLL developing in some people living with HTLV-1 infection is currently unknown.
There are four subtypes of ATLL: acute, lymphoma, chronic and smouldering. Each subtype has different clinical presentations.
Incidence
Overall lifetime risk of ATLL in people living with HTLV-1 infection is estimated to be between 2% and 5%. The risk appears to increase with age, longer duration of infection, in people with higher proviral load, and in those with a family history of ATLL. Although a higher proviral load is associated with progression to ATLL there is no clear threshold that can be applied to an individual to reliably predict their risk.
Mortality
The prognosis of untreated ATLL is generally poor but varies considerably by type and between individuals. Four-year survival rates range from 52% for smouldering to 11% for acute type.
Symptoms and Clinical Findings
People with ATLL can present with a range of symptoms depending on the type. The key symptoms and signs include generalised lymphadenopathy, hepatosplenomegaly and skin lesions (nodules, plaques or rash). Blood tests may show elevated white blood cells, hypercalcaemia and elevated LDH. Patients can also present with opportunistic infections (e.g. Pneumocystis jirovecci, candida, cytomegalovirus, and Strongyloides stercoralis) secondary to ATLL induced immunosuppression.
Diagnosis
ATLL is diagnosed using a combination of clinical features and laboratory examination of malignant cells in a person with confirmed HTLV-1 infection.
Treatment
There are a variety of treatment options including chemotherapy with allogeneic hematopoietic stem cell transplantation, antiviral agents and monoclonal antibodies. Most patients do not achieve a cure with current treatment options.
Management and Care
Refer for specialist care including treatment and management of opportunistic infections.
