1/256. Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin.We describe a case of granulomatous mycosis fungoides, tumor stage, mimicking sarcoidosis in an 82-year-old man with a 2-year history of skin disease. The final diagnosis was established after one of seven biopsy specimens showed a nongranulomatous histologic picture of patch-stage mycosis fungoides. Monoclonality was proven for the lymphocytic population by T-cell-receptor rearrangement studies. The unusually extensive granulomatous inflammation with huge giant cells surrounded by CD1a-positive cells in the other six biopsy specimens was suggestive of the histopathology of granulomatous slack skin, another rare granulomatous cutaneous T-cell lymphoma. Because both a clinical and histologic overlap between granulomatous mycosis fungoides and granulomatous slack skin have been reported in the literature, we conclude that they may belong to the spectrum of a single disease.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
2/256. Cutaneous T-cell lymphoma and human immunodeficiency virus infection: 2 cases and a review of the literature.Cutaneous non-Hodgkin's lymphomas are rare in patients with hiv-1 infection and almost all of the cases reported are of T-cell lineage with histopathological features of mycosis fungoides or sezary syndrome. We studied 2 cases of mycosis fungoides in hiv-1-positive patients who were intravenous drug abusers and were in stage II and IV C2 (CDC'86), respectively. The first patient (stage II) had multiple, erythematous and infiltrated large plaques on the abdomen, back, arms and legs, whereas the second patient (stage IV) had smaller erythematous, slightly scaly and infiltrated pruritic plaques on the trunk and limbs. Their CD4 lymphocyte counts were 634 and 250 cells/mm3, respectively. Biopsies showed features consistent with mycosis fungoides, with an epidermotropic pattern. The immunohistochemical study revealed a T-cell lineage of this atypical infiltrate. Both patients partially responded to topical steroid ointment, showing moderate improvement. Further biopsies performed 6 months later confirmed the prior diagnosis of mycosis fungoides. No tumour stage was observed during a 2-year follow-up. We conclude that mycosis fungoides is rare in HIV-positive patients, but must be included in the differential diagnosis of erythematous plaques in these patients. In suspected, but non-diagnostic cases of mycosis fungoides in HIV-positive patients, only a close clinical and histopathological follow-up can confirm the diagnosis.- - - - - - - - - - ranking = 5keywords = lymphoma (Clic here for more details about this article) |
3/256. Systemic sarcoidosis and cutaneous lymphoma: is the association fortuitous?The association of systemic sarcoidosis and malignant lymphoma is known as the 'sarcoidosis-lymphoma syndrome'. Cutaneous involvement is rare in this syndrome. We report a 52-year-old woman who was diagnosed as having tumour-stage mycosis fungoides. Complete remission was achieved by combination therapy consisting of isotretinoin, interferon (IFN) alpha, electron beam irradiation, photochemotherapy and topical corticosteroids. Three years later, the patient developed systemic sarcoidosis characterized by yellowish papules on the abdominal wall and the eyelids that histologically revealed non-caseating granulomas, multiple fine-nodular interstitial pulmonary infiltrates on chest X-ray, hilar lymphadenopathy, decreased vital capacity and increased lymphocyte count in bronchoalveloar lavage fluid. As opposed to most of the reported cases, in our patient the manifestation of cutaneous lymphoma preceded the diagnosis of systemic sarcoidosis. We review the cases reported in the literature and discuss a possible causal and temporal relationship as well as the role of IFN alpha in the development of sarcoidosis.- - - - - - - - - - ranking = 7keywords = lymphoma (Clic here for more details about this article) |
4/256. 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.- - - - - - - - - - ranking = 12keywords = lymphoma (Clic here for more details about this article) |
5/256. 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.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
6/256. Follicular mycosis fungoides.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.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
7/256. 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.- - - - - - - - - - ranking = 9keywords = lymphoma (Clic here for more details about this article) |
8/256. 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.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
9/256. A novel Epstein-Barr virus-like virus, HV(MNE), in a macaca nemestrina with mycosis fungoides.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.- - - - - - - - - - ranking = 3keywords = lymphoma (Clic here for more details about this article) |
10/256. 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.- - - - - - - - - - ranking = 5keywords = lymphoma (Clic here for more details about this article) |
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