Aggressive malignancy of activated T cells associated with human T-cell lymphotrophic virus type-I (HTLV-1).
Classified into smoldering, chronic, lymphoma and acute subtypes.
A retrovirus infecting 5,000,000 to 10,000,000 people worldwide.
T-cell lymphotrophic virus type-I (HTLV-1) can cause an incurable neurologic disorder HTLV-one-associated myelopathy-tropical spastic paraparesis as well as adult T cell leukemia-lymphoma.
Poor prognosis associated with acute subtype and lymphoma subtype because of intrinsic resistance to chemotherapy the presence of a large tumor burden, hypercalcemia, immune deficiencies and the presence of infection.
Aggressive subtypes have not been impacted by chemotherapy on long-term survival rates.
Smoldering and chronic subtypes have a better prognosis.
Combination therapy with antiretroviral agent zidovudine and alpha interferon have reported high response rates in acute disease.
The prevalence HTLV-1 varies geographically with clustered populations in Japan, South America, the Middle East, Africa, and the Caribbean.
HTLV-1 occurs particluarly in sounthern Japanese islands and leds to the development of in aproximately 10% of infected patients
The median age of individuals with HTLV-1 infection is about 65 years, and increasing.
No disease entity linked to HTLV-2.
Risk of transmission in blood transfusion 1 in 3 million.