National Cancer Institute®
Last Modified: October 1, 2002
UI - 12243671
AU - Kontochristopoulos GJ; Exadaktylou D; Hatziolou E; Tassidou A;
TI - Zakopoulou N Follicular mucinosis associated with early stage cutaneous T-cell lymphoma: successful treatment with interferon alpha-2b and acitretin.
SO - J Dermatolog Treat 2001 Jun;12(2):117-21
AD - 2nd Department of Dermatology, A Syngros Hospital, Athens, Greece.
BACKGROUND: Follicular mucinosis (FM) is a rare dermatosis characterized by mucin deposits in the pilosebaceous units. It is divided into a primary-benign type and a secondary type associated mostly with lymphomas. No standard effective therapy is available for the primary FM while in the secondary form treatment is aimed against the underlying disease. METHODS: We report a case of secondary FM in which a cutaneous T-cell lymphoma was detected 6 years after the initial eruption. RESULTS: Complete remission was achieved with combination therapy of interferon alpha-2b at a dose of 6 million U subcutaneously three times a week, and acitretin 35 mg/day, for 6 months. CONCLUSION: Regular clinical and histopathological evaluation is suggested for all patients with FM. For cases associated with cutaneous T-cell lymphoma the combination of interferon alpha and acitretin seems to be a good therapeutical approach.
UI - 12045708
AU - Lukowsky A; Richter S; Dijkstal K; Sterry W; Muche JM
TI - A T-cell receptor gamma polymerase chain reaction assay using capillary electrophoresis for the diagnosis of cutaneous T-cell lymphomas.
SO - Diagn Mol Pathol 2002 Jun;11(2):59-66
AD - Department of Dermatology and Allergy, Medical Faculty (Charite), Humboldt-University of Berlin, Germany. email@example.com
Detection of clonal T-cell receptor gamma rearrangements by polymerase chain reaction (TCRgamma PCR) followed by high-resolution electrophoresis has now become a valuable tool in the diagnosis of cutaneous T-cell lymphoma (CTCL). The identification of clonal TCRgamma PCR products by fluorescent fragment analysis (FFA) on a capillary DNA sequencer is described here and compared with an established hetero-duplex temperature gradient gel electrophoresis (HD-TGGE). Of 55 CTCL derived lesional skin samples, clonality was obtained in 46 samples by FFA (83.6%) and in 45 samples by HD-TGGE (81.8%). Of 35 control skin specimens from various nonmalignant dermatoses, two samples (pityriasis lichenoides chronica) showed clonality by both methods, one sample (chronic dermatitis) only by FFA. The sensitivity of FFA was established using three clonal T-cell lines and peripheral blood mononuclear cells. The detection limit for clonal material was approximately 1% to 2.5% in mixtures of DNA and 1% to 3% in cell dilutions. For cell dilution series, we confirmed a linear correlation between the clonal/polyclonal peak-size ratios and the portion of clonal cells up to about 10%. Thus, the initial ratio between mono-and polyclonal template is correctly displayed by FFA within that concentration range. In conclusion, FFA on capillary DNA sequencer is a well-suited separation technique in TCRgamma PCR-based clonality analysis also exhibiting quantitative properties.
UI - 12196250
AU - Chua MS; Veness MJ
TI - Mycosis fungoides involving the oral cavity.
SO - Australas Radiol 2002 Sep;46(3):336-9
AD - Department of Radiation Oncology, Westmead Hospital, Sydney, Australia.
Mycosis fungoides is a malignant T-cell lymphoproliferative disease with a predilection for cutaneous involvement. Extracutaneous disease is uncommon and oral mucosal involvement is rare. We describe a case of mycosis fungoides involving the hard palate treated with radiotherapy. The relevant literature on this topic is reviewed.
UI - 12224972
AU - Suchin KR; Junkins-Hopkins JM; Rook AH
TI - Treatment of stage IA cutaneous T-Cell lymphoma with topical application of the immune response modifier imiquimod.
SO - Arch Dermatol 2002 Sep;138(9):1137-9
AD - Department of Dermatology, Hospital of the University of Pennsylvania, 2 Rhoads Pavilion, 3600 Spruce St, Philadelphia, PA 19104-2399, USA. firstname.lastname@example.org
UI - 11407974
AU - Willers J; Haffner A; Zepter K; Storz M; Urosevic M; Burg G; Dummer R
TI - The interferon inhibiting cytokine IK is overexpressed in cutaneous T cell lymphoma derived tumor cells that fail to upregulate major histocompatibility complex class II upon interferon-gamma stimulation.
SO - J Invest Dermatol 2001 Jun;116(6):874-9
AD - Department of Dermatology, University Hospital of Zurich, Zurich, Switzerland.
Cutaneous T cell lymphomas are characterized by an accumulation of malignant clonal lymphocytes in the skin and occasionally in the blood. We compared gene transcription profiles from cultured clonal lymphocytes with autologous healthy blood lymphocytes by microarray hybridization. Cutaneous T cell lymphoma derived cells transcribed high amounts of an interferon inhibiting cytokine factor. The presence of an interferon inhibiting cytokine factor was confirmed in 12 skin biopsies of mycosis fungoides and Sezary syndrome derived blood lymphocytes by reverse transcriptase-polymerase chain reaction. The presence of interferon inhibiting cytokine factor mRNA in Sezary syndrome derived lymphocytes was associated with a lack of HLA class II upregulation after stimulation with interferon-alpha and interferon-gamma. This was not due to a loss of the interferon signaling cascade as the presence of interferon-signaling components was confirmed by reverse transcriptase--polymerase chain reaction on the transcriptional level. The elevated constitutive interferon inhibiting cytokine factor expression observed in cutaneous T cell lymphoma derived cells was insensitive to interferon-gamma stimulation, but was enhanced in normal peripheral blood mononuclear cells. We suggest that interferon inhibiting cytokine factor contributes to the lack of HLA class II upregulation in lymphoma cells. Interferon inhibiting cytokine factor may participate in providing a microenvironment at the tumor site insensitive to interferon-gamma stimulation and thus prevents an efficient local immune response.
UI - 12006543
AU - Zhang C; Hazarika P; Ni X; Weidner DA; Duvic M
TI - Induction of apoptosis by bexarotene in cutaneous T-cell lymphoma cells: relevance to mechanism of therapeutic action.
SO - Clin Cancer Res 2002 May;8(5):1234-40
AD - Department of Dermatology, The University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
PURPOSE: Bexarotene is the first synthetic rexinoid approved for the treatment of all stages of cutaneous T-cell lymphoma (CTCL) however the mechanism of bexarotene action is unknown. We examined the effects of bexarotene on induction of apoptosis and expression of its cognate receptors in well-established CTCL cell lines (MJ, Hut78, and HH). EXPERIMENTAL DESIGN: CTCL cells were treated with 0.1, 1, and 10 microM bexarotene for 24, 48, 72, and 96 h. Apoptosis was determined by flow-cytometry analysis of sub-G(1) hypodiploid nuclei and annexin V binding populations. Apoptosis-associated proteins and retinoid receptors were detected by Western blots. RESULTS: Bexarotene treatment at 1 and 10 microM for 96 h increased the number of cells with sub-G1 populations and annexin V binding in a dose-dependent manner compared with vehicle controls (DMSO) in all three cell lines, respectively. Bexarotene treatment suppressed the expression of retinoid X receptor alpha and retinoic acid receptor alpha proteins in all three lines compared with untreated controls. Bexarotene treatment decreased the protein levels of survivin, activated caspase-3, and cleaved poly(ADP-Ribose) polymerase, but had no obvious effect on expression of Fas/Fas ligand and bcl-2 proteins in all three CTCL lines. CONCLUSIONS: Bexarotene treatment at clinically relevant concentrations causes apoptosis of CTCL cell lines in association with activation of caspase-3 and cleavage of poly(ADP-Ribose) polymerase, as well as down-regulation of retinoid X receptor alpha, retinoic acid receptor alpha, and survivin. These findings support apoptosis as a mechanism for bexarotene therapy in CTCL.
UI - 11859306
AU - Rampino M; Ragona R; Monetti U; Mussano A; Guarneri A; Sannazzari GL
TI - Total skin electron beam therapy in mycosis fungoides. Our experience from 1985 to 1999.
SO - Radiol Med (Torino) 2002 Jan-Feb;103(1-2):108-14
AD - Dipartimento di Radioterapia, Universita degli Studi, Turin, Italy.
PURPOSE: The specific goal of this retrospective study is to evaluate the role of total skin electron beam therapy (TSEBT) in the treatment of Mycosis Fungoides (MF) and to assess the most significant prognostic factors in univariate and multivariate analyses. MATERIAL AND METHODS: technique) were performed on a total of 86 patients (63 with Mycosis Fungoides, 6 with Sezary Syndrome and 17 with Cutaneous Lymphomas). This study considers only the Mycosis Fungoides group, which consisted of 60 cases evaluable for response, survival and toxicity. The distribution of patients by stage (MFCG Staging Classification, 1991) was 21, 5, 12, 22 for stages I, II, III and IV, respectively. RESULTS: The overall response rate was 96.6% (58/60) with complete response (CR) in 50/60 patients (83.3%) and partial response (PR) in 8 cases (13.3%). The five-year and ten-year actuarial overall survival (OS) was 50% and 45%, respectively. Local control, intended as control of the disease in the skin, was 35% at five years and 20% at ten years, and was correlated with skin involvement. The prognostic factors confirmed by the multivariate analysis for both overall survival and local control were: T (p<0.001) and response after TSEBT (p<0.001). The treatment was very well tolerated. CONCLUSIONS: Our study showed good results in terms of response and survival with a long follow-up time (mean value 40 months). We confirm that TSEBT yields very good results in early-stage MF; additional trials of combined modality and investigational therapies are needed to improve the outcome for advanced-stage disease.
UI - 12150131
AU - Trupiano JK; Bringelsen K; Hsi ED
TI - Primary cutaneous lymphoblastic lymphoma presenting in an 8-week old infant.
SO - J Cutan Pathol 2002 Feb;29(2):107-12
AD - Department of Pathology, Cleveland Clinic Foundation, OH 44195, USA.
BACKGROUND: We report a case of primary cutaneous lymphoblastic lymphoma (LBL) presenting in an 8-week-old infant. METHODS: Histopathology and flow cytometric analysis confirmed the diagnosis of a lymphoblastic lymphoma. The cells expressed CD19, CD20, CD34 and surface immunoglobulin (sIg). RESULTS: The cells were negative for TdT and CD99. This unusual immunophenotype has been described as "transitional pre-B-cell", in that the cells express both immature markers (CD34) and mature markers (sIg). CONCLUSIONS: To our knowledge, only five other cases of sIg positive precursor B-cell LBL have been reported in the literature. This may represent the youngest reported case of primary cutaneous LBL, occurring at 8weeks of age.
UI - 12271301
AU - Pabsch H; Rutten A; Von Stemm A; Meigel W; Sander CA; Schaller J
TI - Treatment of childhood mycosis fungoides with topical PUVA.
SO - J Am Acad Dermatol 2002 Oct;47(4):557-61
AD - Department of Dermatology, Dermatohistological Unit, St Barbara Hospital, Duisburg, Germany.
Mycosis fungoides is the most common type of cutaneous T-cell lymphoma, which is usually observed in mid to late adulthood. We report 5 cases of mycosis fungoides in children, all presenting as patch- and plaque-stage disease most commonly involving the buttocks. Histologic examination showed in every case the typical features of mycosis fungoides. In 4 of the 5 cases, the infiltrating lymphocytes were characterized by the T-cell phenotype CD3(+), CD4(+), CD8(+); and in 3 cases, a monoclonal rearrangement of the T-cell receptor gamma (TCR-gamma) gene was found. Three children received topical PUVA treatment, and the other two were treated with mid-potency topical corticosteroids, resulting in complete clinical remission. A management approach to mycosis fungoides with topical PUVA may be appropriate for children.
UI - 12271309
AU - Koay J; Jones D; Duvic M
TI - Cold urticaria in a patient with mycosis fungoides.
SO - J Am Acad Dermatol 2002 Oct;47(4):608-10
AD - Baylor College of Medicine, Houston, Texas 77030, USA.
We report what we believe to be the first documentation of a patient with both cold urticaria and mycosis fungoides. The patient described a marked worsening of his long-standing lesions of mycosis fungoides at the same time as the onset of cold sensitivity. We believe this suggests a possible association between these 2 rare diseases.
UI - 12271315
AU - Shapiro M; Yun M; Junkins-Hopkins JM; Vittorio CC; Schulman N; Saidman
TI - BH; Fried RG; Rook AH; Alavi A Assessment of tumor burden and treatment response by 18F-fluorodeoxyglucose injection and positron emission tomography in patients with cutaneous T- and B-cell lymphomas.
SO - J Am Acad Dermatol 2002 Oct;47(4):623-8
AD - Department of Dermatology, University of Pennsylvania Health System, Philadelphia, USA.
18F-Fluorodeoxyglucose (FDG)-positron emission tomography (PET) is a unique functional/metabolic imaging modality that is efficacious in nodal staging and detection of extranodal involvement for a variety of lymphomas. We report its novel use in evaluating tumor burden and response to therapy in two patients with cutaneous lymphomas. A 24-year-old woman with aggressive subcutaneous panniculitic T-cell lymphoma associated with fever, arthralgias, lymphadenopathy, mild anemia, and widespread painful lesions refractory to multiple treatment strategies exhibited intense uptake of a glucose analogue at sites of clinically apparent (and clinically imperceptible) disease. Denileukin diftitox therapy resulted in clinical remission, and a repeat PET scan failed to detect residual foci of malignancy. A 38-year-old man with a more indolent multifocal primary cutaneous follicle center B-cell lymphoma characterized by few systemic symptoms and slowly evolving lesions demonstrated only mild glucose analogue uptake at sites of disease. Remission was achieved by radiotherapy and intravenous rituximab, and confirmed by a repeat PET scan. Extracutaneous disease was not evident in either patient by this technique. These preliminary data suggest that FDG-PET may be useful in determining disease activity at the time of initial diagnosis, after treatment, and evaluating a suspected recurrence.
UI - 12200382
AU - Ingen-Housz-Oro S; Bussel A; Flageul B; Michel L; Dubertret L; Kourilsky
TI - P; Gachelin G; Bachelez H; Musette P A prospective study on the evolution of the T-cell repertoire in patients with Sezary syndrome treated by extracorporeal photopheresis.
SO - Blood 2002 Sep 15;100(6):2168-74
AD - Institut de Recherche sur la Peau, Institut National de la Sante et de la Recherche Medicale (INSERM) U532, Hopital Saint-Louis, 75475 Paris, France.
Sezary syndrome is a leukemic form of epidermotropic cutaneous T-cell lymphoma related to the malignant proliferation of clonal CD4(+) T cells. Extracorporeal photochemotherapy may induce a transient improvement of the clinical signs, but its efficiency is discussed. To investigate the frequency of the T-cell clone in the peripheral blood of patients with Sezary syndrome and to monitor its evolution in patients treated using extracorporeal photopheresis or chemotherapy, we used the immunoscope technique. In one patient, we observed a decrease of the relative frequency of the clone from 15.6% to 0%, paralleling a complete remission of the clinical disease and a disappearance of the circulating Sezary cells. In the other cases, the evolution of the relative frequency paralleled the initial improvement of the clinical status and the absence of long-term efficiency in patients treated with extracorporeal photopheresis or chemotherapy. We observed a quick-acting direct cytotoxicity of the association 8MOP + UVA on the T-cell clone. The immunoscope technique appears to be an efficient tool to appreciate the amount of tumoral cells and to monitor the evolution of the clonal component in the Sezary syndrome.
UI - 12209749
AU - Chiarion-Sileni V; Bononi A; Fornasa CV; Soraru M; Alaibac M; Ferrazzi
TI - E; Redelotti R; Peserico A; Monfardini S; Salvagno L Phase II trial of interferon-alpha-2a plus psolaren with ultraviolet light A in patients with cutaneous T-cell lymphoma.
SO - Cancer 2002 Aug 1;95(3):569-75
AD - Division of Medical Oncology, Azienda Ospedaliera-Universita, Via Giustiniani 2, 35123 Padua, Italy. email@example.com
PURPOSE: To evaluate the efficacy and side effects of psolaren with ultraviolet light A (PUVA) and interferon-alpha-2a (IFN-alpha-2a) in patients with mycosis fungoides (MF) and Sezary syndrome (SS). PATIENTS all stages of MF and SS were treated in a prospective Phase II trial with systemic escalating doses of IFN-alpha-2a combined with PUVA for 1 year, followed by indefinite PUVA maintenance in complete responding patients. RESULTS: Sixty-three patients were enrolled (Stage IA, n = 6; IB, n = 37; IIA, n = 3; IIB, n = 3; III, n = 12; IVA, n = 2). Ten patients had received previous therapy. The median follow-up duration for the entire cohort is 37 months. Of 63 patients, 51 achieved a complete response (CR; 74.6%) or partial response (PR; 6%) to therapy. The median response duration is 32 months. The 5-year overall survival rate is 91% and the 5-year disease-free survival rate is 75%. No life-threatening side effects were observed. Five patients stopped IFN-alpha-2a therapy due to toxicity. Eighty-four percent of the patients received more than 75% of the planned dose (12 million units three times a week). CONCLUSIONS: This combination of IFN-alpha-2a and phototherapy is an effective and safe therapy for patients with symptomatic MF. Copyright 2002 American Cancer Society.
UI - 12218920
AU - De Saint Paul G; Albes B; Bayle P; Lamant L; Bazex J
TI - [Cutaneous T-cell lymphoma, cutaneous hyperpigmentation and paraneoplastic pemphigus]
SO - Ann Dermatol Venereol 2002 Jun-Jul;129(6-7):896-900
AD - Service de Dermatologie, Hopital Nord, Marseille, France.
BACKGROUND: Paraneoplastic pemphigus is an autoimmune mucocutaneous disease usually associated with neoplasia as lymphoid proliferations. We report the original case of a patient who had developed a mycosis fongoide preceded by a paraneoplastic pemphigus. To our knowledge, this association has never been reported before. Cutaneous manifestations of mycosis fongoide as pigmentary change are known. This mycosis fongoide was particular in its progressive cutaneous hyperpigmentation. CASE REPORT: A 70-year-old male patient developed a mycosis fongoide with CD30 positive cells in the dermis several months after the diagnosis of a paraneoplastic pemphigus. Simultaneously, a cutaneous hyperpigmentation was predominantly noticed on photo-exposed areas, which improved after chemotherapy. DISCUSSION: Paraneoplastic pemphigus may precede the cancer, as is shown by our present case. This paraneoplastic pemphigus is singular because of the lack of oral erosions, patient's prolonged survival and its association with a mycosis fongoide. The diagnosis of mycosis fongoide with CD30 + cells was finally established together with its relationship to the cutaneous hyperpigmentation. Indeed, a few cases of pigmentary changes in mycosis fongoide have already been reported. The pathogenesis is still unknown, but the role of mast cell and stem cell factor in hyperpigmented mycosis fongoide has been proposed.
UI - 11893040
AU - Barrionuevo C; Anderson VM; Zevallos-Giampietri E; Zaharia M; Misad O;
TI - Bravo F; Caceres H; Taxa L; Martinez MT; Wachtel A; Piris MA Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru.
SO - Appl Immunohistochem Mol Morphol 2002 Mar;10(1):7-14
AD - Department of Pathology, The Institute of Neoplastic Diseases, Lima, Peru.
Hydroa-like cutaneous T-cell lymphoma (hydroa-like CTCL) is an unusual pediatric malignancy with a poor prognosis. An impressive cutaneous rash characterized by edema, blisters, ulcers, crusts, and scars, resembling hidroa vacciniforme, is seen mainly on the face and sometimes on the extremities. The lesion consists of lymphomatous T-cell infiltration of the skin and subcutis with variable exocytosis and angiocentricity. It has been also called edematous, scarring vasculitic panniculitis and hydroa-like lymphoma. An association with Epstein-Barr virus has been suggested. The differential diagnosis includes other cutaneous lymphomas, particularly the cutaneous nasal type T/natural killer-cell lymphoma, mycosis fungoides, precursor T-cell lymphoblastic lymphoma, nonspecific peripheral T-cell lymphoma, cutaneous anaplastic large cell lymphoma, and subcutaneous panniculitic T-cell lymphoma. Other differential diagnoses are inflammatory dermatopathies and panniculitides. Based on a series of 16 such cases referred to the Institute of Neoplastic Diseases, the objective of this report is not only to provide a better clinicopathologic understanding of this entity but also a reappraisal of it as a malignancy. The male/female frequency ratio was 1:1. The median age was 10 years old. All cases showed predominant facial involvement with edema, blisters, ulcers, crusts, and scars. Chemotherapy and/or radiotherapy had little or no benefit. The prognosis was usually dismal. The lymphoma extended from the epidermis to the subcutis, with frequent angiocentric and periadnexal array. Lymphoma cells were mostly of intermediate size with dense hyperchromatic nuclei, inconspicuous nucleoli, and infrequent mitosis. A scanty and variable inflammatory background was found. The lymphoma cells displayed T-cell cytotoxic phenotype. In addition, they were negative for the natural killer cell antigens CD56 and CD57. Epstein-Barr virus in situ hybridization was positive in the six cases in which it was assayed. T-cell receptor gamma (TCRgamma) displayed monoclonal-type rearrangement in four cases studied. Our findings indicate that hydroa-like CTCL is an independent clinicopathologic entity that affects children. Consequently, it should be considered an independent subset of CTCLs and be included as such in the classification of neoplastic diseases of the lymphoid tissues.
The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.