Adult T-cell Leukemia/Lymphoma (ATLL) is a T-cell neoplasm occurring in subjects whose CD4+ T-cells have been infected by retrovirus HTLV-I (Human T-lymphotropic virus type-I).
In patients with the acute or leukemic type of ATLL the disease progresses rapidly. This tumor is highly responsive to combination chemotherapy, however the response is transient and ATLL relapses within few months after remission in most cases. On the other hand, there is a relatively prolonged course, exceeding 2 years, in chronic type of ATLL. A French Group reported successful results using chemotherapy, zidovudine and interferon in a series of 5 cases [1].
ATLL has been reported worldwide but areas of high incidence include Japan, Central and South America, Iran, West and Central Africa and Melanesia [2]. The cumulative lifetime risk of developing ATLL is 2% among HTLV-I – infected patients, with >95% of affected patients showing serologic evidence of HTLV-I. Yet there is no vaccine, no means of assessing the risk of disease or prognosis in infected people [2].
There are some reports of familial ATLL [3,4] in the world and this is the first occurrence in Iran. There appear to be host factors that affect transformation of lymphocytes by HTLV-I, and evidence suggests that there may be hostrelated genetic factors [5]. So in this familial case the genetic predisposition may have a great role in development of ATLL from a previous HTLV-I infection. Indeed, household contact and the risk of horizontal transmission of HTLV-I cannot be underestimated.
Although the true prevalence of HTLV-I infection in Iran is still unknown, in most of the reports infected patients arise from northeastern part of this country [6,7]. Presence of these reports stressed the need for a proper study on the prevalence of HTLV-I antibodies in this geographic area.
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