![]() ![]() A bone marrow aspirate, biopsy, and cytogenetics are performed at diagnosis. The disease has a predilection for bone and skin therefore, a full skin exam is performed on all patients and a skeletal survey is ordered on select patients with bone symptoms, fractures or unexplained elevated alkaline phosphatase. The variety and heterogeneity of organ involvement in ATL is such that standard lymphoma staging studies, and additional ATL-specific staging studies should be done on diagnosis ( Table 2). There is no standard treatment for ATL guidelines recommend that patients be enrolled in clinical trials whenever possible. Allogeneic stem cell transplant can significantly prolong survival. Recent work suggests that a subset of ATL patients may benefit from antiviral therapy alone without chemotherapy. The most common presentations of ATL are the acute and lymphoma type which are highly aggressive and have a poor prognosis with survival measured in 6–10 months even with aggressive chemotherapy. This heterogeneous presentation of ATL contributes to the clinical challenges of diagnosing and caring for ATL patients. Patients with ATL present with chronic or acute disease, with a remarkable array of organ involvement seen. Outcomes may vary based on region of origin, with Caribbean ATL reported to have lower survival among patients diagnosed in the USA and socio-economic factors. ![]() Much of the clinical data has been obtained in Japan or the Middle East, where the viral subtype compositions differ from the Caribbean. HTLV-I infects an estimated 10 to 20 million people worldwide and is primarily transmitted by breast feeding, although spread via blood transfusion-although very rare nowadays, sharing of needles, and sexual intercourse also occurs. As it is rare and has a variable clinical presentation, ATL is likely under-diagnosed in the USA. Only 140 US cases have been documented in the SEER database between 19. The incidence of ATL is strongly correlated to the seropositivity of HTLV-1 of the birth place, and common in people originating from Japan, the Caribbean basin, Central and South America, Western Africa, Iran and Southeast USA. During the long latency period the HTLV-1 virus undergoes genetic changes, with paired blood and nodal samples from ATL patients often showing different genome profiles. ATL develops in a small fraction (4% to 5%) of HTLV-1 infected patients several decades after primary infection. ![]()
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