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At least 883 records · Page 49Linked to original sources
PubMed2001

"High-dose" UVA1 therapy of widespread plaque-type, nodular, and erythrodermic mycosis fungoides.

BACKGROUND: UVA1 (340 to 400 nm) was found to be effective in the treatment of early-stage mycosis fungoides (MF). OBJECTIVE: The purpose of this study was to assess the efficacy of UVA1 phototherapy for widespread plaque-type, nodular, and erythrodermic MF. METHODS: Thirteen patients (8 with stage IB, 4 with IIB, and 1 with III MF) received 100 J/cm(2) UVA1 daily until remission. Four patients also had lesions inaccessible by UVA1 that were considered control lesions. Immunocytologic studies of skin infiltrates and circulating T cells were done before and after the therapy. RESULTS: Eleven patients showed complete clinical and histologic responses. Two patients had a partial improvement. Unirradiated control lesions never improved. Serious short-term side effects were not recorded. Circulating CD4(+)/CD45RO(+) and CD4(+)/CD95(+) lymphocytes were significantly reduced by the therapy. CONCLUSION: UVA1 therapy is an effective and well-tolerated treatment for advanced MF. The therapeutic relevance of the effects on circulating lymphocytes remains to be established because lesions in nonexposed cutaneous areas did not respond.

Adult↗
PubMed2001

Evaluation of a new paraffin-reactive CD7 T-cell deletion marker and a polymerase chain reaction-based T-cell receptor gene rearrangement assay: implications for diagnosis of mycosis fungoides in community clinical practice.

BACKGROUND: T-cell deletion and T-cell receptor (TCR) gene rearrangement studies are helpful in the early diagnosis and subsequent management of mycosis fungoides (MF). However, this often requires fresh-frozen tissue that can be difficult to obtain and evaluate in community clinical practice. A new CD7 antibody, the most sensitive and specific T-cell deletion marker, and a new TCR-gamma gene rearrangement polymerase chain reaction (PCR) assay (TCR-gamma PCR) are now available on routine paraffin-embedded biopsy specimens. OBJECTIVE: Our purpose was to assess the utility of CD7 deletion and TCR-gamma PCR in the diagnosis of MF using routine paraffin-embedded biopsy material. METHODS: Cases of MF (n = 17) with matching frozen tissue immunohistochemistry and benign reactive dermatoses (lichen planus; n = 27) were assessed for CD7 (Clone: CD7-272) deletion and TCR-gamma PCR using paraffin-embedded biopsy specimens. RESULTS: Excellent concordance comparing frozen and paraffin embedded CD7 immunostaining (88%) was observed. CD7 deletion and TCR-gamma PCR was sensitive (94%) and specific (96%) for a diagnosis of MF using paraffin-embedded biopsy specimens. CONCLUSION: In the diagnosis of MF, detection of CD7 deletion and monoclonal TCR rearrangements can be successfully performed in a cost-effective, timely fashion using routine formalin-fixed paraffin-embedded biopsy specimens.

Adult↗
PubMed2002

Narrowband UVB phototherapy for early-stage mycosis fungoides.

BACKGROUND: Narrowband UVB (NB-UVB) phototherapy has been shown to be effective for the treatment of various dermatoses. OBJECTIVE: We evaluated the effect of NB-UVB in the treatment of early stage mycosis fungoides (MF). METHODS: The response of 24 patients (12 stage IA, 12 stage IB) with patch stage MF to thrice weekly NB-UVB was assessed. Twelve patients had skin phototypes I-III, and 12 had types IV-VI. Seven patients had hypopigmented MF. Mean follow-up period was 29.0 weeks. RESULTS: Thirteen patients (54.2%) had a complete response (CR), 7 (29.2%) had partial response (PR), and 4 (16.7%) had no response. Specimens from a repeat biopsy in 10 patients with CR showed histologic clearing in 9 of them. Upon discontinuation of treatment, 4 patients with CR relapsed, with a mean time to relapse of 12.5 weeks. CONCLUSION: NB-UVB is a viable, comparably safe, and easily administered alternative in the management of early stage MF.

Adult↗
PubMed2002

Induction of complete remission of advanced stage mycosis fungoides by allogeneic hematopoietic stem cell transplantation.

Advanced mycosis fungoides (MF) is incurable with conventional treatments. High-dose chemoradiotherapy with autologous bone marrow transplantation has induced remissions in a small number of patients with MF, but this modality is limited by a high relapse rate. We report induction of complete remission in a 37-year-old woman with rapidly progressive stage IV MF with allogeneic stem cell transplantation (Allo SCT). She remains in continuous complete remission 2 years after transplant. Allo SCT for MF is theoretically attractive, because there is no contamination of the graft by malignant cells, and because of the possibility of graft-versus-tumor effect. Although the results in this patient are encouraging, more patients and longer follow-up are needed to define the usefulness of Allo SCT in the treatment of MF.

Adult↗
PubMed2002

Total skin electron radiation in the management of mycosis fungoides: Consensus of the European Organization for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Project Group.

Radiotherapy has been successfully implemented in the treatment of mycosis fungoides (MF) for almost a century. With the development of the modern linear accelerator, it has become possible to treat extended areas of the skin with accelerated electrons. Total skin electron beam radiation (TSEB) has been in use for several decades, and a number of technical modifications have been made with the goals of optimizing dose distribution and improving clinical outcome. Emerging evidence from recent studies suggests an association between TSEB techniques and efficacy in the treatment of MF. Based on this evidence, the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group, in association with experts from radiotherapy centers in North America, has reached a consensus on acceptable methods and clinical indications for TSEB in the treatment of MF. The aims of this report are to enhance accessibility of this highly efficacious treatment modality to patients with MF and to provide a point of reference for further clinical research.

Decision Making↗
PubMed2002

Mycosis fungoides: the great imitator.

A considerable number of reports have documented mycosis fungoides (MF) mimicking other dermatoses, but a comprehensive review has not been published. Our aim was to comprehensively review reports of various dermatoses simulated by MF. Additionally, 2 cases in which MF simulated diseases not previously documented (psoriasis and erythema annulare centrifugum) are presented. A literature search of all case reports of MF cited in the MEDLINE database from 1966 through 2000 plus those in one of the author's (H. Z.) files was performed. A total of 23 reported cases of dermatoses mimicked by MF were found. With the additional 2 dermatoses now presented, this yields a total of at least 25 diseases that can be simulated by MF. In view of the considerable number of dermatoses simulated by MF, the term the great imitator is appropriate for MF.

Adult↗
PubMed2002

Increased serum immunoglobulin levels are common in mycosis fungoides and Sézary syndrome.

BACKGROUND: Patients with cutaneous T-cell lymphoma (CTCL; mycosis fungoides [MF] and Sézary syndrome [SS]) acquire immunodeficiency and opportunistic infections. OBJECTIVE: We attempted to determine whether abnormalities of humoral immunoglobulin levels are present. METHODS: A retrospective analysis of serum immunoglobulin levels in patients with CTCL at baseline evaluation at a cancer center was compared to levels in patients with leukemias and levels in healthy control subjects. RESULTS: A total of 254 of 650 patients with CTCL evaluated between 1987 and 2001 had baseline quantitative immunoglobulin levels. Mean IgG, IgA, and IgM levels were similar among all MF/SS patients versus controls. The percentages of MF/SS patients with elevated levels of each immunoglobulin class were higher than percentages in healthy controls, and elevated IgA levels occurred among late versus early patients (P =.043). CONCLUSION: High immunoglobulin levels are more frequent in patients with MF and SS than in healthy controls, patients with chronic lymphocytic leukemia, and patients with hairy cell leukemia. High IgA levels are more frequent in late stage MF/SS.

Adolescent↗
PubMed2003

Narrowband UVB and psoralen-UVA in the treatment of early-stage mycosis fungoides: a retrospective study.

BACKGROUND: Treatment of early-stage mycosis fungoides (MF) consists of topical steroids, phototherapy (UVB), photochemotherapy (psoralen plus UVA [PUVA]), topical nitrogen mustard, or total skin electron-beam irradiation. It has been reported that the same effective UVB dose is safer than PUVA regarding carcinogenicity and produces fewer side effects. Narrowband UVB (311 nm) results in less irritation and erythema and is more effective compared with broadband UVB. OBJECTIVE: Our purpose in this retrospective study was to analyze the response to treatment, relapse-free interval, and irradiation dose in 56 patients with early-stage MF (stage Ia and Ib). A total of 21 patients were treated with narrowband UVB (311 nm); 35 patients were treated with PUVA. RESULTS: Narrowband UVB treatment led to complete remission in 17 of 21 patients (81%), partial remission in 4 of 21 (19%), and none showed progressive disease. PUVA treatment led to complete remission in 25 of 35 patients (71%), partial remission in 10 of 35 (29%), and none showed progressive disease. The mean relapse-free interval for patients treated with UVB was 24.5 months (range, 2-66 months) and for patients treated with PUVA, 22.8 months (range, 1-43 months). CONCLUSION: Narrowband UVB therapy for patients with early-stage MF is an effective treatment modality. It has several advantages over treatment with broadband UVB and PUVA. When treating patients with early-stage MF it may be beneficial to start with narrowband UVB therapy and, if there is progression or no response, switch to PUVA therapy.

Adult↗
PubMed2003

Hypopigmented mycosis fungoides in Caucasian patients: a clinicopathologic study of 7 cases.

BACKGROUND: Hypopigmented mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma. It is more frequent in dark-skinned or Asian patients, particularly children. Only 9 cases in Caucasian patients have been reported in the literature so far. OBSERVATION: We describe 7 Caucasian patients (2 children and 5 adults) with hypopigmented MF. Histologic examination confirmed the diagnosis in all cases. The phenotype of neoplastic lymphocytes was T helper in 4 cases and T suppressor in 3 (2 of them children). Monoclonality of the T lymphocytes could be detected in hypopigmented lesions in all 7 cases with the use of a polymerase chain reaction technique. In 4 patients, polymerase chain reaction analysis of T-cell receptor-gene rearrangement after laser-based microdissection of the specimen revealed that the monoclonal population of T lymphocytes was confined mainly to the epidermis. CONCLUSION: Hypopigmented lesions of MF can be observed in Caucasian patients. Children affected by MF often present with this rare clinical variant of the disease. Persistent or unusual hypopigmented lesions should be subjected to biopsy to avoid delay in the diagnosis of MF, especially in children.

Adolescent↗
PubMed2003

Lymphomatoid papulosis associated with mycosis fungoides: a study of 21 patients including analyses for clonality.

BACKGROUND: Although an association of lymphomatoid papulosis (LyP) with mycosis fungoides (MF) is recognized, our understanding of this relation is limited. OBJECTIVE: We sought to document the clinical experience at the University of California, San Francisco, in 21 patients who had both LyP and MF and to do clonality studies in 7 of those patients in whom this was possible. METHODS: We conducted chart review of the 21 patients and analysis for T-cell receptor-gamma gene rearrangements by the polymerase chain reaction. RESULTS: Of 54 patients, 21 (39%) with LyP had associated MF. LyP preceded MF in 14 (67%), MF preceded LyP in 4 (19%), and there was concurrent appearance in 3 (14%). Of the 21 patients, 20 (95%) were type A and only 1 (5%) was type B. An identical clone was found in lesions of both LyP and MF in all 7 patients in whom analysis was possible. CONCLUSION: Findings of this study strengthen the idea that LyP and MF are related T-cell lymphoproliferative disorders.

Adolescent↗
PubMedDate not supplied

Photodynamic therapy with topical 5-aminolevulinic acid for mycosis fungoides: clinical and histological response.

There is no curative treatment for mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma. The aim of this study was to investigate the response of single lesions to photodynamic therapy (PDT). The study included 10 plaque MF lesions and 2 tumour MF lesions from 10 patients. First, 20% 5-aminolevulinic acid was applied topically to the lesion and adjacent skin for 5-6 h. The lesion was then exposed to red light at around 630nm. Skin biopsies were taken before treatment, after clinical improvement and after clinical remission. The expression of CD3, CD4, CD7, CD8, CD1a, CD34, CD68, CD71, Ki-67, bcl-2 and p53 was studied immunohistochemically. There was complete clinical clearance in seven of nine plaque lesions. Neither tumour lesion responded to PDT. The biopsies confirmed a regression of the infiltrate after treatment. In the sparse remaining infiltrate a few CD4+ and CD8+ cells were found, most of which showed normal bcl-2. There were also fewer proliferating cells, illustrated by a decrease in Ki-67 and CD71. In conclusion, PDT has good clinical and histological effects in treating local plaque MF lesions.

Administration, Topical↗
PubMed2002

Total skin electron irradiation in mycosis fungoides: comparison between a modified Christie Hospital translational technique and the Stanford technique.

Seventy-one patients with mycosis fungoides (MF) were treated by Total skin electron irradiation (TSEI) using either a modified Christie Hospital translational technique (44 pts) or a six dual-field Stanford technique (27 pts). There was no statistical difference in response rate, disease-free survival and overall survival between the two irradiation techniques. However, the Stanford technique was significantly less toxic than the modified Christie Hospital technique.

Adult↗
PubMed2002

Hypomagnesemia and hypocalcemia in mycosis fungoides: a retrospective case series.

The most common variant of cutaneous T-cell lymphomas (CTCLs) is mycosis fungoides (MF), a malignant proliferation of atypical CD4+ CD45Ro+ T-cells that initially proliferate in the skin and later invades lymph nodes and other organs including the blood (Sezary syndrome). The pathogenesis of CTCL is largely unknown. We present definitive data showing a correlation between degree and prevalence of hypomagnesemia and hypocalcemia and clinical stage of MF. Hypomagnesemia was present in 22.2% of MF patients with early stage (n = 27), 38.5% of intermediate stage (n = 13), and 67.5% of advanced stage disease (n = 40). Hypocalcemia was present in 8.3% of MF patients with early stage (n = 24), 54.5% with intermediate stage (n = 11), and 61.0% with advanced stage disease (n = 41). The odds ratios for having hypomagnesemia and hypocalcemia in patients with stage II or higher MF compared with stage I patients were 5.33 and 16.24, respectively. Hypomagnesemia has been associated with immune function abnormalities including the development of T-cell leukemia-lymphoma in rats. We hypothesize that the hypomagnesemia may play a role in the progression or pathogenesis of MF or the Sezary syndrome (SS). This retrospective case series is the first study ever to report a relationship between serum cations and CTCL in humans.

Adult↗
PubMed2001

Mycosis fungoides in pregnancy: remission after treatment with alpha-interferon in a case refractory to conventional therapy: a case report.

Mycosis fungoides complicating pregnancy is rarely encountered. As it is a form of cutaneous T-cell lymphoma, some of the treatment options are contraindicated in pregnancy, and the disease may become unresponsive to safer conventional therapies. We report a patient who, in her third trimester of pregnancy, failed to respond to the treatment options available. Left with little choice, a trial of alpha-interferon was undertaken. The patient responded with remission, and prolongation of pregnancy was achieved.

Adult↗
PubMed1994

Persistence of human T cell lymphotropic virus type 1 (HTLV-1) sequences in peripheral blood mononuclear cells from patients with mycosis fungoides.

Mycosis fungoides (MF) is a rare form of cutaneous T cell lymphoma suspected of having a viral etiology. As in adult T cell leukemia, the virus involved may be human T lymphotropic virus type 1 (HTLV-1). We cultured the peripheral blood mononuclear cells (PBMC) of 29 patients with MF HTLV-1 seronegative by enzyme-linked immunosorbent assay and Western blot. The presence of reverse transcriptase (RT) and p24 antigen was investigated in the concentrate supernatant of the culture. The DNA of all studied patients was submitted to polymerase chain reaction and Southern blot analysis using primers and probes recognizing the tax region of HTLV-1/2 and the pol region of HTLV-1. 10 of 29 patients were found positive to HTLV-1, whereas they were always negative to RT and p24. The same results were confirmed in double blind after 6 mo. Our findings suggest HTLV-1 may be involved in the etiology of MF, at least in certain cases.

Adult↗
PubMed1994

Cutaneous disease resembling mycosis fungoides in HIV-infected patients whose skin and blood cells also harbor proviral HTLV type I.

Two homosexual HIV-infected patients with lymphocyte counts of < 50 presented with intense pruritus, hyperpigmentation, and skin lesions clinically suggestive of the cutaneous T cell lymphoma, mycosis fungoides. On light microscopy, the skin biopsies were difficult to interpret because of the sparseness of the lymphocytic infiltrates. However, electron microscopy revealed typical Sézary cells in the peripheral blood and skin. Cultures of blood mononuclear cells of one of the patients generated HTLV-I-like particles. Although both patients lacked antibodies to HTLV, their blood and skin specimens proved to harbor tax and pol HTLV-I proviral sequences as shown by the polymerase chain reaction and Southern blot analysis. Dual infection with HIV and HTLV should be considered in the diagnostic work-up of patients at risk, even in the absence of demonstrable antibodies. Dual infections could result in clinical manifestations and evolution of disease not anticipated in patients who harbor only one of these retroviruses.

AIDS-Related Opportunistic Infections↗
PubMed1994

Flow cytometric detection of neoplastic T cells in patients with mycosis fungoides based on levels of T-cell receptor expression.

The authors report the flow cytometric detection of neoplastic T cells in the peripheral blood of four out of five (80%) patients with peripheral blood involvement with mycosis fungoides (Sezary syndrome) based on the levels of T-cell receptor expression as measured by CD3 and TCR-alpha beta staining. Antigen receptor expression was abnormal in terms of increased density of surface CD3 or TCR-alpha beta per cell. Other immunophenotypic abnormalities were present in three of these patients. However, in one patient abnormal T-cell receptor expression was the only immunophenotypic evidence of neoplasia, although morphologically abnormal lymphocytes were present and a T-cell clone was detected by polymerase chain reaction (PCR). In another patient, the authors were able to detect development of a new, more aggressive neoplastic T-cell population based on levels of T-cell receptor expression. Levels of T-cell receptor expression may be of diagnostic utility in the evaluation of peripheral blood for the presence of neoplastic T-cell populations.

Aged↗
PubMed1984

Mycosis fungoides/Sezary's syndrome progressing to immunoblastic sarcoma. A T-cell lymphoproliferation with both helper and suppressor phenotypes.

Multiparameter studies of an unusual patient exhibiting cutaneous manifestations of both mycosis fungoides and Sezary's syndrome are presented. The neoplastic cells of dermal and nodal infiltrates and peripheral blood expressed both helper and suppressor immunologic phenotypes. Cytofluorographic analysis of cells isolated from lymph node and peripheral blood showed a population of neoplastic cells that were stained with the monoclonal antibodies OKT 3, 4, 8, and 11. Immunoperoxidase staining of frozen sections with monoclonal antibodies Leu 1, 2, and 3 provided a topographical identification of an identically marking population of cells in dermis and lymph node. In light of current models depicting normal T-cell lineage, the authors suggest that the neoplastic population in this patient, expressing both helper and suppressor phenotypes, reflected a phenotypic stage of immunologic maturation (OKT 6-, OKT 10-, OKT 3+, OKT 11+, OKT 4+, OKT 8+) in which the neoplastic cells had not yet segregated into distinctive T-cell subsets. While excess helper activity was suggested by serum hypergammaglobulinemia, in vitro helper and suppressor function was not determined. The range of studies employed illustrates the wide variety of technics required to adequately characterize complex clinico-immunopathologic disorders, as represented by this case, and the wealth of information that can be gleaned.

Antibodies, Monoclonal↗