RESUMEN
An 88-year-old Inuit man from Northern Canada presented with an extensive skin rash associated with numerous violaceous skin nodules on his palms and lower extremities. Biopsy of a skin nodule revealed Kaposi's sarcoma (KS), a human herpesvirus 8 (HHV8)-associated malignancy, whereas biopsy of the erythematous skin showed an atypical infiltrate of CD4-positive T-cells that, together with TCR gene rearrangement and presence of clonal T-cells in peripheral blood by flow cytometry, was consistent with a T-cell lymphoma, mycosis fungoides (MF) subtype. Serology was negative for HIV and HTLV-I/II and no immunodeficiency syndrome was identified. The patient was successfully treated with an oral retinoid for KS, and with topical hydrocortisone and ultraviolet B (UVB) phototherapy for MF. This case highlights the existence of HHV8-related lesions in native persons of Northern Canada, and also that MF-induced immunosuppression combined with immunosenescence may play a role in the development of non-HIV-related KS.
Asunto(s)
Inuk , Micosis Fungoide/patología , Neoplasias Primarias Múltiples/patología , Sarcoma de Kaposi/patología , Neoplasias Cutáneas/patología , Acitretina/uso terapéutico , Administración Cutánea , Anciano de 80 o más Años , Antiinflamatorios/uso terapéutico , Herpesvirus Humano 8 , Humanos , Hidrocortisona/uso terapéutico , Huésped Inmunocomprometido , Inmunosenescencia , Masculino , Micosis Fungoide/inmunología , Micosis Fungoide/terapia , Neoplasias Primarias Múltiples/inmunología , Neoplasias Primarias Múltiples/terapia , Sarcoma de Kaposi/tratamiento farmacológico , Sarcoma de Kaposi/etnología , Sarcoma de Kaposi/inmunología , Neoplasias Cutáneas/inmunología , Neoplasias Cutáneas/terapia , Terapia Ultravioleta/métodosRESUMEN
The simultaneous occurrence of two different lymphomas in a 57-year-old white woman is reported: mycosis fungoides and a leukemic lymphoplasmacytoid immunocytoma. The first was confirmed by histologic study and electron microscopy, and the latter by histologic study and immunoperoxidase staining. The lymphoid cells in the involved bone marrow and peripheral blood expressed the same surface immunoglobulin as was found in the cytoplasm of the immunocytoma cells, i.e., IgM-lambda. The clonal B-cell expansion was brought into a lasting remission by chlorambucil, but the cutaneous lymphoma proved to be refractory to therapy. The patient died 38 months after diagnosis.