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1. Scarisbrick JJ: Staging and management of cutaneous T-cell lymphoma. Clin Exp Dermatol; 2006 Mar;31(2):181-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Most variants of CTCL are indolent lymphoma, the most common being mycosis fungoides.
  • In addition, Sézary syndrome, the leukaemic variant, has an aggressive clinical course.
  • The tumour, node, metastasis and blood stage needs to be documented and used to determine an overall stage from IA to IVB.
  • Treatment of patients with early-stage disease (IA-IIB) should be limited to skin-directed therapy.
  • More advanced or resistant disease may be treated with systemic therapies such as extracorporeal photopheresis, immunotherapy, monoclonal antibody therapy, novel retinoids or chemotherapy, and where possible, patients should be entered into clinical trials.
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Bone Marrow Transplantation / methods. Dermatologic Agents / therapeutic use. Humans. Immunotherapy. Mycosis Fungoides / pathology. Mycosis Fungoides / therapy. Photopheresis / methods. Phototherapy / methods. Sezary Syndrome / classification. Sezary Syndrome / pathology. Sezary Syndrome / therapy

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  • (PMID = 16487086.001).
  • [ISSN] 0307-6938
  • [Journal-full-title] Clinical and experimental dermatology
  • [ISO-abbreviation] Clin. Exp. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Dermatologic Agents
  • [Number-of-references] 34
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2. Apisarnthanarax N, Talpur R, Duvic M: Treatment of cutaneous T cell lymphoma: current status and future directions. Am J Clin Dermatol; 2002;3(3):193-215
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of cutaneous T cell lymphoma: current status and future directions.
  • The treatment of cutaneous T cell lymphoma (CTCL), which includes mycosis fungoides and Sezary syndrome, has been in a state of continual change over recent decades, as new therapies are constantly emerging in the search for more effective treatments for the disease.
  • However, prognosis and survival of patients with CTCL remains dependent upon overall clinical stage (stage IA-IVB) at presentation, as well as response to therapy.
  • Past therapies have been limited by toxicity or the lack of consistently durable responses, and few treatments have been shown to actually alter survival, especially in the late stages of disease.
  • Even aggressive chemotherapy has not been shown to improve overall survival compared to conservative sequential therapy in advanced disease, and adds the risk of immunosuppressive complications.
  • Over the last decade, extracorporeal photopheresis has been the only single treatment that has been shown to improve survival in patients with Sezary syndrome, although its true efficacy and place in combination therapy remain unclear.
  • Much of the focus of current research has been on combinations of skin-directed therapies and biological response modifiers, which improve response rates.
  • The results of various trials over the years have also brought into favor the use of post-remission maintenance therapy with topical corticosteroids, topical mechlorethamine (nitrogen mustard), interferon-alpha, or phototherapy to prevent disease relapse.
  • Recent novel developments in CTCL therapy include oral bexarotene, a retinoid X receptor-selective retinoid that has activity in all stages of CTCL, and the topical gel formulation of bexarotene, which plays a role in treating localized lesions.
  • US Food and Drug Administration (FDA)-approved, oral systemic bexarotene has the advantage of a 48% overall response rate at a dosage of 300 mg/m(2)/day, and avoids immunosuppression and risk of central line and catheter-related infectious complications that are associated with other systemic therapies.
  • Denileukin diftitox is associated with capillary leak syndrome in 20 to 30% of patients, which may be ameliorated by hydration and corticosteroids.
  • Higher response rates are possible by combining bexarotene with "statin" drugs and active CTCL therapies.
  • Biological response modifier therapies that are in current or future investigational trials include topical tazarotene, pegylated interferon, interleukin-2, and interleukin-12.
  • At the forefront of systemic chemotherapy development, pegylated liposomal doxorubicin, gemcitabine, and pentostatin appear to have the greatest potential for success in CTCL therapy.
  • [MeSH-major] Lymphoma, T-Cell, Cutaneous / therapy
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Antibodies, Monoclonal / therapeutic use. Antineoplastic Agents / therapeutic use. Bone Marrow Transplantation. Cytokines / therapeutic use. Humans. Interferons / therapeutic use. Mechlorethamine / therapeutic use. PUVA Therapy. Photopheresis. Retinoids / therapeutic use. Ultraviolet Therapy

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  • (PMID = 11978140.001).
  • [ISSN] 1175-0561
  • [Journal-full-title] American journal of clinical dermatology
  • [ISO-abbreviation] Am J Clin Dermatol
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 16672-22; United States / NCI NIH HHS / CA / K24 CA 86815; United States / NCI NIH HHS / CA / R21 CA 74117
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] New Zealand
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Antibodies, Monoclonal; 0 / Antineoplastic Agents; 0 / Cytokines; 0 / Retinoids; 50D9XSG0VR / Mechlorethamine; 9008-11-1 / Interferons
  • [Number-of-references] 241
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3. Ure UB, Ar MC, Salihoglu A, Guner SI, Baran A, Oguz O, Ferhanoglu B: Alemtuzumab in Sézary syndrome: efficient but not innocent. Eur J Dermatol; 2007 Nov-Dec;17(6):525-9
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  • [Title] Alemtuzumab in Sézary syndrome: efficient but not innocent.
  • Mycosis fungoides is the most common form of cutaneous T-cell lymphomas.
  • The related Sézary syndrome is a more aggressive form in which the skin is diffusely affected and the peripheral blood is involved.
  • Although easily managed during its early phases, late-stage mycosis fungoides/Sézary syndrome is usually difficult to treat and becomes refractory to chemotherapy.
  • Recently, promising case-based results have been obtained with alemtuzumab, a humanized immunoglobulin G1 monoclonal antibody that binds to CD52 cell surface antigens, in the treatment of advanced stage mycosis fungoides/Sézary syndrome.
  • We report a case of Sézary syndrome treated successfully with alemtuzumab but who died of treatment-related infection.
  • [MeSH-major] Antibodies, Monoclonal / adverse effects. Antibodies, Neoplasm / adverse effects. Antineoplastic Agents / adverse effects. Cytomegalovirus Infections / chemically induced. Lymphopenia / chemically induced. Opportunistic Infections / chemically induced. Sezary Syndrome / drug therapy

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  • (PMID = 17951134.001).
  • [ISSN] 1167-1122
  • [Journal-full-title] European journal of dermatology : EJD
  • [ISO-abbreviation] Eur J Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antigens, CD; 0 / Antigens, Neoplasm; 0 / Antineoplastic Agents; 0 / CD52 antigen; 0 / DNA, Viral; 0 / Glycoproteins; 3A189DH42V / alemtuzumab
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4. Kuzel TM: Systemic chemotherapy for the treatment of mycosis fungoides and Sézary syndrome. Dermatol Ther; 2003;16(4):355-61
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  • [Title] Systemic chemotherapy for the treatment of mycosis fungoides and Sézary syndrome.
  • The treatment of mycosis fungoides and Sézary syndrome is unique.
  • The treatments of choice are highly stage-and practitioner-dependent.
  • For early stage patients, treatment with palliative topical therapies is often adequate to yield excellent, high-quality and durable responses.
  • It has been hypothesized that some of our therapies work through this mechanism, such as photopheresis and perhaps even psoralen and ultraviolet A.
  • Because of the above, many practitioners have actively discouraged the use of chemotherapy because it may impair the host immune system; certainly, the purine analogs would fall into this category.
  • As the present authors will detail in this paper, the advances in understanding cancer biology and mechanisms of resistance should, in the future, lead to optimal selection of agents that are predicted to be optimally active, limiting the toxicity and waste associated with ineffective drug usage.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Mycosis Fungoides / drug therapy. Sezary Syndrome / drug therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Clinical Trials as Topic. Dose-Response Relationship, Drug. Drug Administration Schedule. Female. Humans. Infusions, Intravenous. Male. Neoplasm Staging. Prognosis. Risk Assessment. Survival Analysis. Treatment Outcome


5. Health Quality Ontario: Extracorporeal photophoresis: an evidence-based analysis. Ont Health Technol Assess Ser; 2006;6(6):1-82
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  • OBJECTIVE: To assess the effectiveness, safety and cost-effectiveness of extracorporeal photophoresis (ECP) for the treatment of refractory erythrodermic cutaneous T cell lymphoma (CTCL) and refractory chronic graft versus host disease (cGvHD).
  • The most frequently diagnosed form of CTCL is mycosis fungoides (MF) and its leukemic variant Sezary syndrome (SS).
  • The relative frequency and disease-specific 5-year survival of 1,905 primary cutaneous lymphomas classified according to the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification (Appendix 1).
  • Mycosis fungoides had a frequency of 44% and a disease specific 5-year survival of 88%.
  • Sezary syndrome had a frequency of 3% and a disease specific 5-year survival of 24%.
  • Cutaneous T cell lymphoma has an annual incidence of approximately 0.4 per 100,000 and it mainly occurs in the 5(th) to 6(th) decade of life, with a male/female ratio of 2:1.
  • Mycosis fungoides is an indolent lymphoma with patients often having several years of eczematous or dermatitic skin lesions before the diagnosis is finally established.
  • Mycosis fungoides commonly presents as chronic eczematous patches or plaques and can remain stable for many years.
  • Early in the disease biopsies are often difficult to interpret and the diagnosis may only become apparent by observing the patient over time.
  • A particular syndrome in these patients involves erythroderma (intense and usually widespread reddening of the skin from dilation of blood vessels, often preceding or associated with exfoliation), and circulating tumour cells.
  • CHRONIC GRAFT VERSUS HOST DISEASE: Allogeneic hematopoietic cell transplantation (HCT) is a treatment used for a variety of malignant and nonmalignant disease of the bone marrow and immune system.
  • The procedure is often associated with serious immunological complications, particularly graft versus host disease (GvHD).
  • The syndrome typically develops several months after transplantation, when the patient may no longer be under the direct care of the transplant team.
  • Of the patients with extensive disease, approximately 60% will respond to treatment and eventually be able to discontinue immunosuppressive therapy.
  • The remaining patients will develop opportunistic infection, or require prolonged treatment with immunosuppressive agents.
  • The syndrome typically occurs 4 to 7 months after transplantation but may begin as early as 2 months or as late as 2 or more years after transplantation.
  • The onset of the syndrome may be abrupt but is frequently insidious with manifestations evolving gradually for several weeks.
  • The most commonly involved tissues are the skin, liver, mouth, and eyes.
  • TREATMENT:   CUTANEOUS T CELL LYMPHOMA: The optimal management of MF is undetermined because of its low prevalence, and its highly variable natural history, with frequent spontaneous remissions and exacerbations and often prolonged survival.
  • Nonaggressive approaches to therapy are usually warranted with treatment aimed at improving symptoms and physical appearance while limiting toxicity.
  • Given that multiple skin sites are usually involved, the initial treatment choices are usually topical or intralesional corticosteroids or phototherapy using psoralen (a compound found in plants which make the skin temporarily sensitive to ultraviolet A) (PUVA).
  • "Second line" therapy for early stage disease is often topical chemotherapy, radiotherapy or total skin electron beam radiation (TSEB).
  • Treatment of advanced stage (IIB-IV) MF usually consists of topical or systemic therapy in refractory or rapidly progressive SS.
  • Bone marrow transplantation and peripheral blood stem cell transplantation have been used to treat many malignant hematologic disorders (e.g., leukemias) that are refractory to conventional treatment.
  • Reports on the use of these procedures for the treatment of CTCL are limited and mostly consist of case reports or small case series.
  • CHRONIC GRAFT VERSUS HOST DISEASE: Patients who develop cGvHD require reinstitution of immunosuppressive medication (if already discontinued) or an increase in dosage and possibly addition of other agents.
  • The current literature regarding cGvHD therapy is less than optimal and many recommendations about therapy are based on common practices that await definitive testing.
  • Numerous salvage therapies have been considered in patients with refractory cGvHD, including ECP.
  • Due to uncertainty around salvage therapies, Bhushan and Collins suggested that ideally, patients with refractory cGvHD should be entered into clinical trials.
  • Two Ontario expert consultants jointly estimated that there may be approximately 30 new erythrodermic treatment resistant CTCL patients and 30 new treatment resistant cGvHD patients per year who are unresponsive to other forms of therapy and may be candidates for ECP.
  • Extracorporeal photopheresis is a procedure that was initially developed as a treatment for CTCL, particularly SS.
  • The lymphocyte layer is treated with methoxsalen (a drug that sensitizes the lymphocytes to light) and exposed to UVA, following which the lymphocytes are returned to the patient.
  • Photosensitization is achieved by administering methoxsalen to the patient orally 2 hours before the procedure, or by injecting methoxsalen directly ino the leucocyte rich fraction.
  • The latter approach avoids potential side effects such as nausea, and provides a more consistent drug level within the machine.
  • In general, from the time the intravenous line is inserted until the white blood cells are returned to the patient takes approximately 2.5-3.5 hours.
  • For CTCL, the treatment schedule is generally 2 consecutive days every 4 weeks for a median of 6 months.
  • For cGvHD, an expert in the field estimated that the treatment schedule would be 3 times a week for the 1(st) month, then 2 consecutive days every 2 weeks after that (i.e., 4 treatments a month) for a median of 6 to 9 months.
  • REGULATORY STATUS: The UVAR XTS Photopheresis System is licensed by Health Canada as a Class 3 medical device (license # 7703) for the "palliative treatment of skin manifestations of CTCL."
  • It is not licensed for the treatment of cGvHD.

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  • (PMID = 23074497.001).
  • [Journal-full-title] Ontario health technology assessment series
  • [ISO-abbreviation] Ont Health Technol Assess Ser
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC3379535
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6. Akita Y, Watanabe D, Yanagishita T, Kuhara T, Kawamura C, Masuda Y, Kawada M, Nakaseko H, Tamada Y, Matsumoto Y: The effect of psoralen plus ultraviolet A in vitro in HUT-78 enhances by 5-aminolevulinic acid. Photodermatol Photoimmunol Photomed; 2007 Apr-Jun;23(2-3):95-7
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  • BACKGROUND: Sezary syndrome and mycosis fungoides are forms of cutaneous T-cell lymphoma, and in the early stage of these diseases psoralen plus ultraviolet A (PUVA) is one of the treatments of choice.
  • Photodynamic therapy using 5-aminolevulinic acid (ALA-PDT) is an effective, non-invasive, and safe treatment for most superficial skin cancers.
  • RESULTS: In our results, cell proliferation compared with controls was inhibited to 53.2% with UVA alone, 52.3% with 1 microM 8-MOP, 43.8% with 100 microM ALA, and 19.2% with combined 8-MOP and ALA.
  • Combined treatment with ALA-PDT and PUVA using a UVA lamp appears to have a strong treatment effect.
  • [MeSH-major] Aminolevulinic Acid / administration & dosage. Methoxsalen / administration & dosage. PUVA Therapy. Photosensitizing Agents / administration & dosage. Skin Neoplasms / drug therapy
  • [MeSH-minor] Cell Line, Tumor / drug effects. Cell Line, Tumor / radiation effects. Cell Proliferation / drug effects. Cell Proliferation / radiation effects. Drug Synergism. Humans. Mycosis Fungoides / drug therapy. Mycosis Fungoides / pathology. Sezary Syndrome / drug therapy. Sezary Syndrome / pathology. Ultraviolet Rays

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  • (PMID = 17523931.001).
  • [ISSN] 0905-4383
  • [Journal-full-title] Photodermatology, photoimmunology & photomedicine
  • [ISO-abbreviation] Photodermatol Photoimmunol Photomed
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Photosensitizing Agents; 88755TAZ87 / Aminolevulinic Acid; U4VJ29L7BQ / Methoxsalen
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7. Scarisbrick JJ, Child FJ, Clift A, Sabroe R, Whittaker SJ, Spittle M, Russell-Jones R: A trial of fludarabine and cyclophosphamide combination chemotherapy in the treatment of advanced refractory primary cutaneous T-cell lymphoma. Br J Dermatol; 2001 May;144(5):1010-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A trial of fludarabine and cyclophosphamide combination chemotherapy in the treatment of advanced refractory primary cutaneous T-cell lymphoma.
  • METHODS: Nine patients with erythrodermic CTCL were identified for the study, eight of whom met the criteria for Sézary syndrome (SS), and three with tumour-stage mycosis fungoides (MF).
  • Six patients had treatment withdrawn, five due to bone marrow suppression and one due to progressive disease.
  • No difference in pretrial parameters were found in those who had treatment withdrawn and those who tolerated at least three courses.
  • As with other multiagent chemotherapy regimens, bone marrow toxicity is a common and severe side-effect.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Lymphoma, T-Cell, Cutaneous / drug therapy. Skin Neoplasms / drug therapy. Vidarabine / analogs & derivatives
  • [MeSH-minor] Aged. Cyclophosphamide / administration & dosage. Female. Follow-Up Studies. Humans. Middle Aged. Mycosis Fungoides / drug therapy. Pilot Projects. Sezary Syndrome / drug therapy. Treatment Outcome


8. Bouwhuis SA, Davis MD, el-Azhary RA, McEvoy MT, Gibson LE, Knudsen JM, Kist JM, Pittelkow MR: Bexarotene treatment of late-stage mycosis fungoides and Sézary syndrome: development of extracutaneous lymphoma in 6 patients. J Am Acad Dermatol; 2005 Jun;52(6):991-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Bexarotene treatment of late-stage mycosis fungoides and Sézary syndrome: development of extracutaneous lymphoma in 6 patients.
  • Bexarotene is a retinoid drug that is approved for the treatment of cutaneous T-cell lymphoma.
  • We report 6 cases in which the initiation of bexarotene therapy for cutaneous T-cell lymphoma was temporally associated with the progression of internal disease despite improvement in cutaneous signs and symptoms.
  • Although bexarotene therapy may alleviate symptoms and signs of cutaneous T-cell lymphoma, careful surveillance of lymph nodes and solid organs during treatment is advised.
  • [MeSH-major] Antineoplastic Agents / adverse effects. Lymphoma, T-Cell / chemically induced. Mycosis Fungoides / drug therapy. Sezary Syndrome / drug therapy. Skin Neoplasms / drug therapy. Tetrahydronaphthalenes / adverse effects

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  • [CommentIn] J Am Acad Dermatol. 2006 Aug;55(2):365-6; author reply 366 [16844539.001]
  • [CommentIn] J Am Acad Dermatol. 2009 Aug;61(2):361-2 [19615550.001]
  • (PMID = 15928617.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Tetrahydronaphthalenes; A61RXM4375 / bexarotene
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9. Pulini S, Rupoli S, Goteri G, Pimpinelli N, Alterini R, Tassetti A, Scortechini AR, Offidani M, Mulattieri S, Stronati A, Brandozzi G, Giacchetti A, Mozzicafreddo G, Ricotti G, Filosa G, Bettacchi A, Simonacci M, Novelli N, Leoni P: Pegylated liposomal doxorubicin in the treatment of primary cutaneous T-cell lymphomas. Haematologica; 2007 May;92(5):686-9
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  • [Title] Pegylated liposomal doxorubicin in the treatment of primary cutaneous T-cell lymphomas.
  • Pegylated liposomal doxorubicin (Peg-Doxo) is a promising drug for advanced/recalcitrant primary cutaneous T-cell lymphomas (CTCLs).
  • We observed overall and complete response rates of 84.2% and 42.1% (with no significant differences between stage I-IIA and IIB-IV patients), and 11% grade III/IV toxicity.
  • Peg-Doxo seems to be an active and safe principle that should be used in plurirelapsed, early stage-MF and in combination with other chemotherapeutic agents in advanced and aggressive CTCLs.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Doxorubicin / analogs & derivatives. Lymphoma, T-Cell, Cutaneous / drug therapy. Polyethylene Glycols / therapeutic use
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Kaplan-Meier Estimate. Liposomes / administration & dosage. Male. Middle Aged. Mycosis Fungoides / drug therapy. Prospective Studies. Remission Induction. Salvage Therapy. Sezary Syndrome / drug therapy. Survival Analysis. Survival Rate. Treatment Outcome

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  • (PMID = 17488695.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Liposomes; 0 / liposomal doxorubicin; 30IQX730WE / Polyethylene Glycols; 80168379AG / Doxorubicin
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10. Horwitz SM, Olsen EA, Duvic M, Porcu P, Kim YH: Review of the treatment of mycosis fungoides and sézary syndrome: a stage-based approach. J Natl Compr Canc Netw; 2008 Apr;6(4):436-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Review of the treatment of mycosis fungoides and sézary syndrome: a stage-based approach.
  • The NCCN Clinical Practice Guidelines in Oncology: Non-Hodgkin's Disease were recently revised to include recommendations for treating mycosis fungoides and Sézary syndrome.
  • Unlike the other forms of non-Hodgkin's lymphomas, stage overwhelmingly determines prognosis and defines radically different treatment approaches.
  • For patients with early-stage disease, initial treatment with skin-directed therapies is preferred, and many patients never require systemic therapy.
  • For patients with refractory or advanced-stage disease, biologic therapies are often the first choices, whereas chemotherapies are reserved for later in the disease course.
  • Many milder therapies may be repeated several times in the disease course, and maintenance and tapering strategies are common.
  • [MeSH-major] Mycosis Fungoides / diagnosis. Mycosis Fungoides / therapy. Sezary Syndrome / diagnosis. Sezary Syndrome / therapy. Skin Neoplasms / diagnosis. Skin Neoplasms / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Hematopoietic Stem Cell Transplantation. Humans. Neoplasm Staging. PUVA Therapy. Prognosis

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  • (PMID = 18433609.001).
  • [ISSN] 1540-1405
  • [Journal-full-title] Journal of the National Comprehensive Cancer Network : JNCCN
  • [ISO-abbreviation] J Natl Compr Canc Netw
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 30
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11. Lundin J, Hagberg H, Repp R, Cavallin-Ståhl E, Fredén S, Juliusson G, Rosenblad E, Tjønnfjord G, Wiklund T, Osterborg A: Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood; 2003 Jun 1;101(11):4267-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome.
  • This phase 2 study evaluated the safety and efficacy of alemtuzumab in 22 patients with advanced mycosis fungoides/Sézary syndrome (MF/SS).
  • Most patients had stage III or IV disease, reduced performance status, and severe itching.
  • Itching, self-assessed on a 0 to 10 visual analog scale, was reduced from a median of 8 before treatment to 2 at end of therapy.
  • Median time to treatment failure was 12 months (range, 5-32+ months).
  • [MeSH-major] Antibodies, Monoclonal / administration & dosage. Antibodies, Neoplasm / administration & dosage. Antineoplastic Agents / administration & dosage. Mycosis Fungoides / drug therapy. Sezary Syndrome / drug therapy
  • [MeSH-minor] Adult. Aged. Antibodies, Monoclonal, Humanized. Humans. Middle Aged. Opportunistic Infections / chemically induced. Pruritus / drug therapy. Remission Induction. Survival Analysis. Treatment Outcome

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  • (PMID = 12543862.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antineoplastic Agents; 3A189DH42V / alemtuzumab
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12. Prince HM, Whittaker S, Hoppe RT: How I treat mycosis fungoides and Sézary syndrome. Blood; 2009 Nov 12;114(20):4337-53
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] How I treat mycosis fungoides and Sézary syndrome.
  • The most common subtypes of primary cutaneous T-cell lymphomas are mycosis fungoides (MF) and Sézary syndrome (SS).
  • Management of MF/SS should use a "stage-based" approach; treatment of early-stage disease (IA-IIA) typically involves skin directed therapies that include topical corticosteroids, phototherapy (psoralen plus ultraviolet A radiation or ultraviolet B radiation), topical chemotherapy, topical or systemic bexarotene, and radiotherapy.
  • Systemic approaches are used for recalcitrant early-stage disease, advanced-stage disease (IIB-IV), and transformed disease and include retinoids, such as bexarotene, interferon-alpha, histone deacetylase inhibitors, the fusion toxin denileukin diftitox, systemic chemotherapy including transplantation, and extracorporeal photopheresis.
  • Examples of drugs under active investigation include new histone deacetylase inhibitors, forodesine, monoclonal antibodies, proteasome inhibitors, and immunomodulatory agents, such as lenalidomide.
  • It is appropriate to consider patients for novel agents within clinical trials if they have failed front-line therapy and before chemotherapy is used.
  • [MeSH-major] Mycosis Fungoides / therapy. Sezary Syndrome / therapy. Skin Neoplasms / therapy
  • [MeSH-minor] Antineoplastic Agents. Combined Modality Therapy. Humans. Radiotherapy

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  • (PMID = 19696197.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 131
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13. Vang R, Medeiros LJ, Malpica A, Levenback C, Deavers M: Non-Hodgkin's lymphoma involving the vulva. Int J Gynecol Pathol; 2000 Jul;19(3):236-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Two patients had neoplasms localized to the vulva, and two patients had a history of NHL that secondarily involved the vulva; in another patient the stage was unknown, and the sixth patient had stage IVA mycosis fungoides/Sezary syndrome involving the vulva.
  • Each tumor was classified according to the revised European-American classification of lymphoid neoplasms: four were diffuse large B-cell lymphoma, one was peripheral T-cell lymphoma, and one was mycosis fungoides/Sezary syndrome.
  • Two patients were treated with chemotherapy and radiotherapy, one patient received chemotherapy and phototherapy, one patient was treated with chemotherapy, and in two patients the treatment is unknown.
  • One patient with low-stage NHL responded to therapy, but relapsed and died of disease 2 years later.
  • The patient with mycosis fungoides/Sezary syndrome is alive with disease at 4 years.
  • [MeSH-minor] Aged. Antigens, CD20 / analysis. Antigens, CD3 / analysis. Female. Humans. Immunohistochemistry. Lymphoma, B-Cell / pathology. Mycosis Fungoides / pathology. Neoplasm Staging. Sezary Syndrome / pathology. Skin Neoplasms / pathology. Vulva / pathology

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  • (PMID = 10907172.001).
  • [ISSN] 0277-1691
  • [Journal-full-title] International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists
  • [ISO-abbreviation] Int. J. Gynecol. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Antigens, CD20; 0 / Antigens, CD3
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14. Guitart J, Wickless SC, Oyama Y, Kuzel TM, Rosen ST, Traynor A, Burt R: Long-term remission after allogeneic hematopoietic stem cell transplantation for refractory cutaneous T-cell lymphoma. Arch Dermatol; 2002 Oct;138(10):1359-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Allogeneic hematopoietic stem cell transplantation has proved to be an effective therapeutic option in various hematologic neoplastic disorders.
  • Because patients with advanced cutaneous T-cell lymphoma have a poor prognosis, with minimal possibilities of sustained remission, we studied the therapeutic potential of hematopoietic stem cell transplantation.
  • OBSERVATIONS: Three young patients with refractory tumor stage mycosis fungoides underwent allogeneic HLA-matched sibling transplantation with combined marrow and CD34-enriched peripheral blood stem cell transplantation after cytoreductive chemotherapy and total-body irradiation.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation / methods. Lymphoma, T-Cell, Cutaneous / pathology. Lymphoma, T-Cell, Cutaneous / therapy
  • [MeSH-minor] Adult. Biopsy, Needle. Female. Follow-Up Studies. Humans. Male. Mycosis Fungoides / pathology. Mycosis Fungoides / therapy. Remission Induction. Severity of Illness Index. Sezary Syndrome / pathology. Sezary Syndrome / therapy. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 12374543.001).
  • [ISSN] 0003-987X
  • [Journal-full-title] Archives of dermatology
  • [ISO-abbreviation] Arch Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 28
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15. Duvic M: Systemic monotherapy vs combination therapy for CTCL: rationale and future strategies. Oncology (Williston Park); 2007 Feb;21(2 Suppl 1):33-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Systemic monotherapy vs combination therapy for CTCL: rationale and future strategies.
  • There are few approved therapies for cutaneous T-cell lymphoma (CTCL).
  • For patients with early-stage disease, the oral retinoids can be combined with other therapies, such as psoralen plus ultraviolet A or interferon alpha, to improve response rates.
  • Combined-modality therapy with oral retinoids, combined chemotherapy, electron-beam therapy, and topical mustargen has also proved effective.
  • For the treatment of advanced-stage disease, the targeted therapy denileukin diftitox (Ontak) provides a nonimmunosuppressive alternative to conventional chemotherapy or radiation therapy.
  • This agent can be used in combination with a maintenance therapy of bexarotene (Targretin) to manage the plaques and patches of mycosis fungoides.
  • Several other targeted therapies are now also in testing, for example, alemtuzumab (CamPath), HuMax-CD4, several histone deacetylase inhibitors, and the transition-state inhibitor forodesine.
  • These drugs, in combination with currently used therapies, may increase the number and combinations of therapies available for the treatment of this chronic condition to optimize long-lasting responses in CTCL.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Lymphoma, T-Cell, Cutaneous / drug therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Antibodies, Monoclonal / therapeutic use. Antibodies, Monoclonal, Humanized. Antibodies, Neoplasm / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant / trends. Diphtheria Toxin / therapeutic use. Dose-Response Relationship, Drug. Humans. Hydroxamic Acids / therapeutic use. Immunologic Factors / therapeutic use. Interferon-alpha / therapeutic use. Interleukin-2 / therapeutic use. Mechlorethamine / therapeutic use. Purine Nucleosides / therapeutic use. Pyrimidinones / therapeutic use. Radiotherapy, Adjuvant / trends. Recombinant Fusion Proteins / therapeutic use. Retinoids / therapeutic use. Sezary Syndrome / drug therapy

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  • (PMID = 17474358.001).
  • [ISSN] 0890-9091
  • [Journal-full-title] Oncology (Williston Park, N.Y.)
  • [ISO-abbreviation] Oncology (Williston Park, N.Y.)
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antineoplastic Agents; 0 / Diphtheria Toxin; 0 / Hydroxamic Acids; 0 / Immunologic Factors; 0 / Interferon-alpha; 0 / Interleukin-2; 0 / Purine Nucleosides; 0 / Pyrimidinones; 0 / Recombinant Fusion Proteins; 0 / Retinoids; 25E79B5CTM / denileukin diftitox; 3A189DH42V / alemtuzumab; 426X066ELK / forodesine; 50D9XSG0VR / Mechlorethamine; 58IFB293JI / vorinostat
  • [Number-of-references] 58
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16. Dummer R, Foss F, Dreno B, Bagot M: Clinical experience: practical management of five patients with cutaneous T-cell lymphoma (CTCL)-related symptoms. Semin Oncol; 2006 Feb;33(1 Suppl 3):S26-32
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • There are now a wide variety of therapeutic options for managing patients with cutaneous T-cell lymphoma-related symptoms.
  • These include skin-directed therapies such as psoralen with UVA irradiation (PUVA), topical chemotherapies such as mechlorethamine (nitrogen mustard) and carmustine (BCNU), electron beam radiation, and systemic therapies such as chemotherapy, photopheresis, and interferons.
  • Although treatment algorithms exist for patients with early stage disease, often treatments are individualized, based on patient specific factors, cost, and accessibility of referral centers for specialized therapies.
  • [MeSH-major] Dermatitis, Exfoliative / drug therapy. Erythema Nodosum / drug therapy. Lymphoma, T-Cell, Cutaneous / drug therapy. Mycosis Fungoides / drug therapy. Sezary Syndrome / drug therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Aged. Antineoplastic Agents / therapeutic use. Carmustine / therapeutic use. Combined Modality Therapy. Female. Ficusin / administration & dosage. Humans. Male. Middle Aged. PUVA Therapy. Phototherapy

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  • (PMID = 16516673.001).
  • [ISSN] 0093-7754
  • [Journal-full-title] Seminars in oncology
  • [ISO-abbreviation] Semin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; KTZ7ZCN2EX / Ficusin; U68WG3173Y / Carmustine
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