11/555. Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma. PURPOSE: To determine the frequency of ophthalmic abnormalities in patients with cutaneous T-cell lymphoma (mycosis fungoides and sezary syndrome) and T-cell lymphoma involving the skin and to describe the clinical course of the disease with selected examples. methods: A computerized diagnostic retrieval system was used to identify all patients with T-cell lymphoma involving the skin who were examined at the Mayo Clinic (Rochester, minnesota) between January 1, 1976 and December 31, 1990. The medical records of affected patients were reviewed. RESULTS: During the 15-year interval from 1976 through 1990, cutaneous T-cell lymphoma was diagnosed in 2,155 patients. Of these 2,155 patients, 42 (1.95%; 26 male and 16 female) had at least 1 ophthalmic abnormality attributable to the disease. The diagnoses in these 42 patients were mycosis fungoides in 19, clinical variants of T-cell lymphoma of the skin (most commonly, peripheral T-cell lymphoma) in 11, and sezary syndrome in 12. Cicatricial eyelid ectropion was the most common finding, affecting 17 (40.4%) of the 42 patients. Thirty-seven patients had findings that, although probably not a direct consequence of cutaneous T-cell lymphoma, have been cataloged in previous studies. CONCLUSION: Although ophthalmic abnormalities in patients with cutaneous T-cell lymphoma are relatively uncommon, the manifestations of the disease are diverse and frequently difficult to treat. ( info) |
12/555. mycosis fungoides with mucinosis follicularis in childhood. mycosis fungoides is a cutaneous T-cell lymphoma (CTCL) usually observed in mid to late adulthood. It occurs only rarely during childhood. Follicular mucinosis is a chronic dermatosis involving the sebaceous glands and outer root sheaths. It is normally differentiated into a juvenile benign form and an adult form possibly associated with mycosis fungoides. We report a 12-year-old boy who presented with an 8-month history of erythematous mucinous plaques on the scalp. Three months later, he developed erythematous patches and plaques on his whole body, accompanied by cervical lymphadenopathy. A biopsy showed follicular mucinosis and epidermotropism of the lymphocytic infiltrate. immunophenotyping and a PCR clonality test were consistent with CTCL. The patient received PUVA treatment and local steroids, resulting in partial remission. mycosis fungoides should be considered in the differential diagnosis of chronic, scaling dermatoses in childhood. Moreover, follicular mucinosis in childhood can be associated with mycosis fungoides. ( info) |
| We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa. ( info) |
14/555. Epidermotropic cutaneous B-cell lymphoma mimicking mycosis fungoides. Cutaneous involvement by B-cell lymphoma is often secondary to systemic disease. Primary cutaneous B-cell lymphomas are less common, and patients generally have an excellent prognosis. We present a patient with cutaneous B-cell lymphoma with clinical and histologic features mimicking mycosis fungoides. Although the patient was initially misdiagnosed as having a T-cell lymphoma, immunophenotypic studies demonstrated that this was a B-cell lymphoma. ( info) |
15/555. mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the literature and study of the clinical course. BACKGROUND: mycosis fungoides (MF) is rare in young patients. Its clinical behavior is still uncertain, as some reports have suggested that it has a more aggressive course than does the adult-onset type. AIM: To ascertain if early-onset MF represents a heterogeneous group of cutaneous T cell lymphomas. MATERIALS AND methods: Clinical, immunohistopathological and follow-up data of early-onset (<20 years of age) MF cases reported in the literature (n = 42) plus 7 described herein were compared with those of a cohort of adult-onset MF patients (n = 252) diagnosed at our institution since 1975. RESULTS: The majority of the 49 early-onset MF patients had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The predominant phenotype was CD3 CD4 CD7-CD8-. Seven patients had a stage progression, 6 with extracutaneous involvement. Five- and 10-year survival rates were 93 and 74%, respectively. CONCLUSIONS: No statistically significant differences were found in the disease course between early- and adult-onset MF. ( info) |
| Epstein-Barr virus (EBV) infection of humans has been associated with the development of lymphoid malignancies mainly of B-cell lineage, although occasionally T-cell lymphomas have been reported. We describe here the characterization of a novel EBV-like virus (HV(MNE)) isolated from a simian T-cell lymphotropic virus type I/II (STLV-I/II) seronegative pigtailed macaque (macaca nemestrina) with a cutaneous T-cell lymphoma. immunohistochemistry studies on the skin lesions demonstrated that the infiltrating cells were of the CD3( )/CD8( ) phenotype. Two primary transformed CD8( ) T-cell lines were obtained from cultures of peripheral blood mononuclear cells (PBMC) and skin, and, with time, both cell lines became interleukin-2-independent and acquired the constitutive activation of STAT proteins. polymerase chain reaction analysis of the dna from the cell lines and tissues from the lymphomatous animal demonstrated the presence of a 536-bp dna fragment that was 90% identical to EBV polymerase gene sequences, whereas the same dna was consistently negative for STLV-I/II sequences. Electron microscopy performed on both cell lines, after sodium butyrate treatment, showed the presence of a herpes-like virus that was designated HV(MNE) according to the existing nomenclature. in situ hybridization studies using EBV Epstein-Barr viral-encoded rna probes showed viral RNA expression in both CD8( ) T-cell lines as well as in the infiltrating CD8( ) T cells of skin-tissue biopsies. Phylogenetic analysis of a 465-bp fragment from the polymerase gene of HV(MNE) placed this virus within the lymphocryptovirus genus and demonstrated that HV(MNE) is a distinct virus, clearly related to human EBV and other EBV-like herpesviruses found in nonhuman primates. ( info) |
17/555. Necrobiotic cutaneous T-cell lymphoma. We report 3 patients with granulomatous cutaneous T-cell lymphoma (CTCL) who showed necrobiosis histologically with palisading granulomas. Although granulomatous change may be present in up to 4% of cases of CTCL, necrobiosis is rare. Misdiagnosis may occur if epidermotropism is minimal or if atypical cells are masked by the granulomatous infiltrate. T-cell receptor gene analysis confirmed the presence of clonal T-cell populations in lesional skin from all 3 cases. ( info) |
| Follicular mycosis fungoides (FMF) is an unusual clinical and histological variant of MF, characterized by selective involvement of hair follicles by atypical lymphocytes. We describe a female patient who had follicular papules located only on the medial aspect of her right thigh. To the best of our knowledge, this patient represents the first reported case of unilesional FMF. ( info) |
| Follicular mycosis fungoides is a rare variant of mycosis fungoides (MF). Structural-wise there are several acneiform lesions made up of comedones, cysts and hyperkeratosis. The main histological finding is atypical lymphocytic infiltration around follicular structures, without epidermotropism. The association with follicular mucinosis is widely discussed in the literature. We report a case of follicular (MF) and review the cases published to date. ( info) |
20/555. Cardiac involvement and molecular staging in a fatal case of mycosis fungoides. polymerase chain reaction (PCR) amplification of T-cell receptor-gamma gene rearrangement was used for molecular staging in a case of primary cutaneous T-cell lymphoma (CTCL) with fatal evolution. Although initial evaluation was negative for systemic involvement, the patient died due to heart failure. autopsy findings revealed lymphomatous myocardial infiltration, but other tissues and organs examined, including lymph nodes, liver, spleen, lung and bone marrow, appeared to be free of disease. Molecular analysis from frozen samples obtained during the initial evaluation, as well as paraffin-embedded material obtained during autopsy, revealed the presence of clonal rearranged bands in all tissues examined except the bone marrow. Subsequent hybridization of PCR products with a tumour-specific oligoprobe confirmed the PCR results, suggesting widespread dissemination of the lymphomatous process. The use of molecular analysis can add significant information about the extent of disease in patients with CTCL and may be helpful in the establishment of therapeutic options. ( info) |