Background: Mycosis fungoides-type cutaneous T-cell lymphoma is uncommon in association with human immunodeficiency virus type 1 (HIV-1) infection. Reported patients have a high incidence of CD8+ T-cell lymphomas and erythroderma, are usually severely immunocompromised, and rapidly die. Chronic, more typical patch-plaque mycosis fungoides-type cutaneous T-cell lymphoma in association with HIV-1 infection has not been reported.
Objective: We report two cases of stage 1B epidermotropic mycosis fungoides-type cutaneous T-cell lymphoma in HIV-1-infected men with no history of the acquired immunodeficiency syndrome.
Methods: Clinical and laboratory data, including lesional immunophenotypic and immunogenotypic findings of two patients with HIV-1-associated cutaneous T-cell lymphoma, were studied.
Results: Immunohistochemical analysis of skin determined the lesional infiltrate of one patient to be CD8+ (Leu-2+), CD7- (Leu-9-), and Leu-8- with germline lesional skin and blood T-cell receptor genes. The other was CD4- (Leu-3+), CD2- (Leu-5-), CD7- (Leu-9-), and Leu-8- with beta-chain T-cell receptor gene rearrangement in lesional skin and blood. Circulating Sézary cells were not detected in either patient. Peripheral CD4/CD8 T lymphocyte numbers did not appear to correlate with cutaneous disease activity or the predominant lesional T-cell subtype. Both patients were responsive to standard therapies and have experienced prolonged survival in excess of that generally reported for HIV-1-associated systemic peripheral and cutaneous T-cell lymphomas.
Conclusion: In the absence of severe immunodeficiency, HIV-1-infected patients with concomitant cutaneous T-cell lymphoma may follow a more typical slowly progressive course.