ABSTRACT
BACKGROUND: Two unusual cases of cutaneous angiocentric T-cell lymphoma were found to be associated with Epstein-Barr virus infection. OBJECTIVE: The objective was to study the clinical course and the response of the disease to conventional chemotherapy. METHODS: Histologic specimens from both patients were studied. Clonal proliferation was assessed by Southern blot hybridization. RESULTS: The disease in both patients was rapidly progressive and responded poorly to aggressive treatment. Biopsy specimens showed infiltration of atypical lymphoid cells with angiocentricity and angiodestruction, which probably resulted in the observed tissue necrosis. Clonal proliferation of Epstein-Barr virus DNA was detected in tissue from primary skin lesions and disseminated nasal lesions. CONCLUSION: Epstein-Barr virus-associated angiocentric T-cell lymphoma in our patients was characterized by an aggressive course and resistance to conventional chemotherapy. A search for Epstein-Barr virus and the human T-lymphotropic virus should be performed in patients with atypical features of cutaneous T-cell lymphoma.
Subject(s)
Herpesvirus 4, Human/isolation & purification , Lymphoma, T-Cell, Cutaneous/microbiology , Skin Neoplasms/microbiology , Adult , Aged , Antibodies, Viral/analysis , Blotting, Southern , Female , Herpesvirus 4, Human/genetics , Herpesvirus 4, Human/immunology , Humans , Immunophenotyping , Karyotyping , Lymphoma, T-Cell, Cutaneous/genetics , Lymphoma, T-Cell, Cutaneous/immunology , Lymphoma, T-Cell, Cutaneous/pathology , Male , Penile Neoplasms/genetics , Penile Neoplasms/immunology , Penile Neoplasms/microbiology , Penile Neoplasms/pathology , Skin/pathology , Skin Neoplasms/genetics , Skin Neoplasms/immunology , Skin Neoplasms/pathologyABSTRACT
We report 11 cases of acute graft-versus-host disease (GVHD); 10 occurred after bone marrow transplantation (BMT) and one following transfusion of nonirradiated whole blood in a patient with severe combined immunodeficiency (SCID). According to the Seattle Classification, 5 cases were of Grade III, 2 Grade II, and 4 Grade I. The skin rash developed between the 6th to 47th day (22 +/- 12 day) after transplantation or transfusion, and was usually manifested initially as erythematous macules or papules in the neck or upper chest. The rash resolved in scaling or became purpuric, pigmented or erythrodermic in severe cases. The grading of skin pathology correlated well with clinical severity of GVHD. Four of the five who had advanced GVHD (Grade III) died. In all 4 cases but one (the SCID case), the skin pathology showed extensive basal vacuolization and multiple dyskeratotic cells. None of the others with mild or focal basal vacuolization and few dyskeratotic cells progressed into advanced GVHD. Three of the 5 cases with advanced GVHD received an underdose of cyclosporin A. These results suggest that severe GVHD carries a poor prognosis and an adequate dose of cyclosporin A is important in alleviating the severity of GVHD and reducing the mortality. The sero-virologic study performed during the skin rash period was negative, suggesting that the skin rash following BMT is mainly caused by GVHD and always precedes intestinal or hepatic manifestations.
