Htlv2induced Diseases

Long-term epidemiological studies in endemic populations have clearly established that HTLV-2 is much less pathogenic in humans than HTLV-1.146 Nevertheless, HTLV-2 has clearly been shown to be associated with clonal T-cell lymphocytoses and has been detected in patients with unusual T-cell neo-plasias.5,147-150 It was first isolated from a cell line derived from the spleen of a patient thought to have T-cell hairy cell leukemia.5 Four years later, a second isolate was obtained from another patient with CD8+ T-cell leukemia and B-cell hairy cell leukemia; the HTLV-2 was monoclonally integrated in the CD8+ leukemia cells but not the B-lymphocytic hairy cells.148 In retrospect, both the patients probably suffered from CD8+ large granular T-lymphocytic (LGL) leukemia. Since then, several groups have identified similar CD8+, CD3+ HTLV-2-infected LGL patients. However, in larger series it is clear that HTLV-2 infection is only rarely associated with this disease.149,150 Also, several HIV-1 and HTLV-2 co-infected patients with a generalized severely pruritic erythrodermatitis have been identified.151-153 Histopathologically, the skin biopsies of these patients have been interpreted as cutaneous T-cell lymphoma (CTCL) and, when examined, the abnormal cells have been shown to be clonal, HTLV-2-infected, CD8+ T lymphocytes. It is unknown whether the natural history of HTLV-2-associated CD8+, T-cell LGL leukemia or CD8+ CTCL differs from that of the HTLV-2 negative forms of these diseases, but patients with both these rare HTLV-associated neoplasms have responded to the conventional treatments usually employed in their viral negative counterparts.

HTLV-2 infection has now been noted in an increasing number of patients with a chronic neurodegenerative disorder.154 This disease is similar to HTLV-1-induced TSP/HAM in that patients have motor deficits, spasticity, and urinary infrequency, albeit to a milder degree. However, in addition, prominent ataxia and altered mental states were present in the HTLV-2-infected patients. These patients also respond to Danazol. HTLV-2 infection has also been associated with an increased susceptibility to cutaneous bacterial infections and immunoglobulin deficiencies. Further studies will be required to define the diseases associated with HTLV-2.

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