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1. Health Quality Ontario: Extracorporeal photophoresis: an evidence-based analysis. Ont Health Technol Assess Ser; 2006;6(6):1-82

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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).
  • BACKGROUND: CUTANEOUS T CELL LYMPHOMA: Cutaneous T cell lymphoma (CTCL) is a general name for a group of skin affecting disorders caused by malignant white blood cells (T lymphocytes).
  • Cutaneous T cell lymphoma is relatively uncommon and represents slightly more than 2% of all lymphomas in the United States.
  • 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%.
  • 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).
  • 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 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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2. Tsatalas C, Martinis G, Margaritis D, Spanoudakis E, Kotsianidis I, Karpouzis A, Bourikas G: Long-term remission of recalcitrant tumour-stage mycosis fungoides following chemotherapy with pegylated liposomal doxorubicin. J Eur Acad Dermatol Venereol; 2003 Jan;17(1):80-2
Hazardous Substances Data Bank. DOXORUBICIN .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Long-term remission of recalcitrant tumour-stage mycosis fungoides following chemotherapy with pegylated liposomal doxorubicin.
  • Advanced stage mycosis fungoides (MF) generally has a poor prognosis, and currently there is no standard treatment available.
  • Here we report the case of a young woman with recalcitrant tumour-stage MF (T3, stage IIb) whose disease was unresponsive to several therapeutic modalities, but who has showed sustained clinical response to pegylated liposomal doxorubucin.
  • The use of this drug in tumour-stage MF should be investigated further.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Doxorubicin / therapeutic use. Mycosis Fungoides / drug therapy. Skin Neoplasms / drug therapy

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  • (PMID = 12602979.001).
  • [ISSN] 0926-9959
  • [Journal-full-title] Journal of the European Academy of Dermatology and Venereology : JEADV
  • [ISO-abbreviation] J Eur Acad Dermatol Venereol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Liposomes; 80168379AG / Doxorubicin
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3. Assaf C: Denileukin diftitox therapy for patients with tumour-stage mycosis fungoides. Dermatol Clin; 2008 Jan;26 Suppl 1:21-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Denileukin diftitox therapy for patients with tumour-stage mycosis fungoides.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Diphtheria Toxin / therapeutic use. Interleukin-2 / therapeutic use. Mycosis Fungoides / drug therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Female. Humans. Middle Aged. Neoplasm Staging. Recombinant Fusion Proteins / therapeutic use

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  • (PMID = 18405182.001).
  • [ISSN] 0733-8635
  • [Journal-full-title] Dermatologic clinics
  • [ISO-abbreviation] Dermatol Clin
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Diphtheria Toxin; 0 / Interleukin-2; 0 / Recombinant Fusion Proteins; 25E79B5CTM / denileukin diftitox
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4. Russell-Jones R, Child F, Olavarria E, Whittaker S, Spittle M, Apperley J: Autologous peripheral blood stem cell transplantation in tumor-stage mycosis fungoides: predictors of disease-free survival. Ann N Y Acad Sci; 2001 Sep;941:147-54
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Autologous peripheral blood stem cell transplantation in tumor-stage mycosis fungoides: predictors of disease-free survival.
  • Nine patients with mycosis fungoides (age range 27-67) underwent autologous peripheral blood stem cell transplantation (PBSCT).
  • All patients had tumor-stage disease, and four had lymph node involvement.
  • Eight patients exhibited a peripheral blood T cell clone using PCR/SSCP analysis of the TCR gamma gene, six prior to harvest and two at the time of harvest.
  • Conditioning prior to reinfusion of stem cells was achieved with various combinations of total skin electron beam (TSEB), total body irradiation (TBI), and chemotherapy, depending upon the patient's prior exposure to radiotherapy.
  • Those with a short DFS were distinguished by rapid tumor onset prior to transplant but not by stage at transplant.
  • Loss of a detectable T cell clone after manipulation of the harvest did not discriminate between the two groups, but rapid relapsers had been subjected to a greater degree of T cell depletion, possibly indicating a compromised cytotoxic response post-PBSCT.
  • The median survival of the cohort is four years from tumor onset, 15 months from PBSCT, and 27 months from the date a peripheral blood clone was first detected in the presence of tumor-stage disease.
  • Our data demonstrate the value of PBSCT for inducing remission in tumor-stage mycosis fungoides.
  • Reinfusion of neoplastic cells could be avoided by harvesting stem cells at an earlier stage in the disease process, preferably before a T cell clone is detectable in the peripheral blood.
  • Alternatively T cell depletion should be restricted to the CD4 subset.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation. Mycosis Fungoides / mortality. Mycosis Fungoides / therapy. Skin Neoplasms / mortality. Skin Neoplasms / therapy
  • [MeSH-minor] Adult. Aged. Cohort Studies. Disease-Free Survival. Genes, T-Cell Receptor gamma. Humans. Lymphocyte Depletion. Middle Aged. Remission Induction. Risk Factors. Transplantation Conditioning. Treatment Outcome

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  • (PMID = 11594568.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Clinical Trial; Journal Article
  • [Publication-country] United States
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5. Steinhoff M, Beyer M, Roewert-Huber J, Lukowsky A, Assaf C, Sterry W: Complete clinical remission of tumor-stage mycosis fungoides after acute extensive skin necroses, granulomatous reaction, and fever under treatment with bexarotene, vorinostat, and high-dose fenofibrate. J Am Acad Dermatol; 2008 May;58(5 Suppl 1):S88-91
Hazardous Substances Data Bank. Fenofibrate .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Complete clinical remission of tumor-stage mycosis fungoides after acute extensive skin necroses, granulomatous reaction, and fever under treatment with bexarotene, vorinostat, and high-dose fenofibrate.
  • Mycosis fungoides and its variants are a distinct entity with a variable, but well-characterized clinical course.
  • We report on a 51-year-old patient with tumor-stage mycosis fungoides who developed several unusual features such as extensive necrosis of lymphoma lesions, granulomatous reaction, and venular thromboses while under treatment with bexarotene, vorinostat, and high-dose fenofibrate.
  • We hypothesize that combination of the high-dose fenofibrate (400 mg) with the retinoid X receptor ligand bexarotene and vorinostat might have induced an increased rate of apoptosis in lymphoma cells in our patient resulting in an extensive release of lymphoma antigens.
  • [MeSH-major] Fenofibrate / administration & dosage. Granuloma / chemically induced. Hydroxamic Acids / administration & dosage. Mycosis Fungoides / drug therapy. Skin Neoplasms / drug therapy. Tetrahydronaphthalenes / adverse effects
  • [MeSH-minor] Acute Disease. Anticarcinogenic Agents / administration & dosage. Anticarcinogenic Agents / adverse effects. Biopsy. Drug Eruptions / pathology. Female. Fever / chemically induced. Humans. Hypolipidemic Agents / administration & dosage. Middle Aged. Necrosis. Remission Induction. Skin / pathology

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  • (PMID = 18489056.001).
  • [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; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anticarcinogenic Agents; 0 / Hydroxamic Acids; 0 / Hypolipidemic Agents; 0 / Tetrahydronaphthalenes; 58IFB293JI / vorinostat; A61RXM4375 / bexarotene; U202363UOS / Fenofibrate
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6. Buhl T, Bertsch HP, Kaune KM, Mitteldorf C, Schön MP, Kretschmer L: Low-dose gemcitabine efficacious in three patients with tumor-stage mycosis fungoides. Clin Lymphoma Myeloma; 2009 Oct;9(5):E21-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Low-dose gemcitabine efficacious in three patients with tumor-stage mycosis fungoides.
  • Mycosis fungoides is the most common subtype of mature T-cell lymphoma that primarily arises in the skin.
  • The tumor manifests as patches, plaques, tumors, or erythroderma and can secondarily involve lymph nodes, peripheral blood, and visceral organs.
  • In advanced tumor stage, chemotherapy is a second-line approach, which is generally not considered curative.
  • Initially, most patients profit from this treatment, but observed remissions usually do not exceed several months.
  • Because of possible immunosuppressive effects in vulnerable patients, the overall benefit of chemotherapy itself is not unequivocal in cutaneous T-cell lymphoma.
  • We report 3 patients whose tumor-stage mycosis fungoides was not sufficiently controlled by several preceding systemic therapies, including liposome-encapsulated doxorubicin.
  • [MeSH-major] Antimetabolites, Antineoplastic / administration & dosage. Deoxycytidine / analogs & derivatives. Mycosis Fungoides / drug therapy. Skin Neoplasms / drug therapy

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  • (PMID = 19858049.001).
  • [ISSN] 1938-0712
  • [Journal-full-title] Clinical lymphoma & myeloma
  • [ISO-abbreviation] Clin Lymphoma Myeloma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0W860991D6 / Deoxycytidine; B76N6SBZ8R / gemcitabine
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7. Markham T, Sheahan K, Collins P: Topical 5-aminolaevulinic acid photodynamic therapy for tumour-stage mycosis fungoides. Br J Dermatol; 2001 Jun;144(6):1262-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Topical 5-aminolaevulinic acid photodynamic therapy for tumour-stage mycosis fungoides.
  • [MeSH-major] Mycosis Fungoides / drug therapy. Photochemotherapy / methods. Skin Neoplasms / drug therapy
  • [MeSH-minor] Aged. Aminolevulinic Acid / therapeutic use. Humans. Male. Photosensitizing Agents / therapeutic use

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  • (PMID = 11422054.001).
  • [ISSN] 0007-0963
  • [Journal-full-title] The British journal of dermatology
  • [ISO-abbreviation] Br. J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Photosensitizing Agents; 88755TAZ87 / Aminolevulinic Acid
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8. 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.
  • BACKGROUND: The combination of fludarabine and cyclophosphamide shows synergistic toxicity in vitro and has been used to treat nodal non-Hodgkin's lymphoma and relapsed chronic lymphocytic leukaemia.
  • OBJECTIVES: To test the efficacy of this combination in 12 patients with cutaneous T-cell lymphoma (CTCL).
  • 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

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  • (PMID = 11359390.001).
  • [ISSN] 0007-0963
  • [Journal-full-title] The British journal of dermatology
  • [ISO-abbreviation] Br. J. Dermatol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 8N3DW7272P / Cyclophosphamide; FA2DM6879K / Vidarabine; P2K93U8740 / fludarabine
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9. Wollina U, Hohaus K, Schönlebe J, Haroske E, Köstler E: Liposomal daunorubicin in tumor stage cutaneous T-cell lymphoma: report of three cases. J Cancer Res Clin Oncol; 2003 Jan;129(1):65-9
Hazardous Substances Data Bank. DAUNORUBICIN .

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  • [Title] Liposomal daunorubicin in tumor stage cutaneous T-cell lymphoma: report of three cases.
  • PURPOSE: Advanced cutaneous T-cell lymphoma (CTCL) is a hard-to-treat condition.
  • The use of liposomal formulation anti-cancer drugs can improve the efficacy and the risk-benefit ratio.
  • Liposomal doxorubicin was shown to be effective as a second-line treatment in CTCL.
  • METHODS: Monotherapy with liposomal-encapsulated daunorubicin (DNX) was given as a monotherapy once a month at 20 mg/m(2) three times to achieve a clinical response.
  • In the case of limited response the drug was given once every 3 weeks and a dose increase was performed.
  • [MeSH-major] Antibiotics, Antineoplastic / administration & dosage. Daunorubicin / administration & dosage. Lymphoma, T-Cell, Cutaneous / drug therapy
  • [MeSH-minor] Adult. Aged. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Drug Administration Schedule. Female. Humans. Liposomes. Male. Treatment Outcome

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  • (PMID = 12618903.001).
  • [ISSN] 0171-5216
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Liposomes; ZS7284E0ZP / Daunorubicin
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10. Di Lucca-Chrisment J, Maubec E, Grossin M, Marinho E, Varet B, Maillard H, Crickx B: [Long-term efficacy of autologous stem cell transplantation for stage IV mycosis fungoides]. Ann Dermatol Venereol; 2009 Nov;136(11):800-5
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  • [Title] [Long-term efficacy of autologous stem cell transplantation for stage IV mycosis fungoides].
  • [Transliterated title] Efficacité a long terme d'une autogreffe de cellules souches au cours d'un mycosis fongoïde de stade IV.
  • BACKGROUND: Mycosis fungoides during large cell transformation to lymphoma has a poor prognosis with mean survival of 36 months.
  • Autologous stem cell transplantation is rarely proposed in this indication.
  • We report the case of a young man still in complete remission for transformed mycosis fungoides 14 years after autologous stem cell transplantation.
  • CASE REPORT: A 25-year-old man presenting eczema-like patches since childhood was treated by chemotherapy for multiple lymphadenopathies considered as Hodgkin's lymphoma.
  • Histology of tumour samples revealed atypical T-cell infiltrate with epidermotropism and presence of more than 25% of large CD30-positive cells.
  • Non-infiltrated patches showed small T-cell lymphoma with epidermotropism.
  • Histological verification of a previous lymphadenopathy confirmed the diagnosis of transformed mycosis fungoides.
  • Despite multiple courses of chemotherapy, the disease progressed, with neurological involvement in particular.
  • Because of tumour aggressiveness, autologous stem cell transplantation was performed and resulted in rapid regression of the tumours, lymphadenopathy and neurological symptoms.
  • Non-transformed mycosis fungoides patches persisted but were controlled with topical mechlorethamine.
  • This patient is still in complete remission for tumour and extracutaneous lesions 14 years after the autograft.
  • DISCUSSION: This was probably a case of juvenile mycosis fungoides diagnosed and transformed in adult age.
  • Neurological involvement by mycosis fungoides is rare and usually carries a drastic prognosis.
  • To our knowledge, this is the longest remission of transformed mycosis fungoides seen after autograft.
  • It highlights the value of this method in aggressive transformed mycosis fungoides, especially in patients ineligible for allograft.
  • [MeSH-major] Mycosis Fungoides / pathology. Mycosis Fungoides / surgery. Stem Cell Transplantation
  • [MeSH-minor] Adolescent. Adult. Biopsy. Humans. Leg / pathology. Male. Skin / pathology. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 19917433.001).
  • [ISSN] 0151-9638
  • [Journal-full-title] Annales de dermatologie et de vénéréologie
  • [ISO-abbreviation] Ann Dermatol Venereol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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11. Barzilai A, Trau H, David M, Feinmesser M, Bergman R, Shpiro D, Schiby G, Rosenblatt K, Or R, Hodak E: Mycosis fungoides associated with B-cell malignancies. Br J Dermatol; 2006 Aug;155(2):379-86
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  • [Title] Mycosis fungoides associated with B-cell malignancies.
  • BACKGROUND: The coexistence of mycosis fungoides, a peripheral T-cell lymphoma, and B-cell malignancies or Hodgkin's lymphoma in the same patient is unusual.
  • OBJECTIVES: To detect cases of mycosis fungoides associated with B-cell malignancies or Hodgkin's lymphoma and to analyse the characteristics of and the interplay between the lymphoproliferative neoplasms.
  • METHODS: Patients with mycosis fungoides who had B-cell malignancies or Hodgkin's lymphoma were selected from among 398 patients either treated or followed up in two tertiary medical centres during a 7-year period.
  • RESULTS: Eleven patients with mycosis fungoides and B-cell malignancy were detected (seven of non-Hodgkin's lymphoma, three of chronic lymphocytic leukaemia, one of multiple myeloma).
  • No case of Hodgkin's lymphoma was found.
  • In seven patients the mycosis fungoides preceded the B-cell malignancy whereas in four it was the B-cell malignancy which occurred first.
  • The time elapsed between onset of the two malignancies ranged from 4 to 22 years (average: 12 years).
  • Patients who had mycosis fungoides as the first neoplasm presented with earlier stages of mycosis fungoides (four of seven: IA, three of seven: IB) than those who had mycosis fungoides as their second neoplasm (of four, one: IB, one: folliculotropic, two: IIB).
  • Among the four patients in whom the appearance of mycosis fungoides followed the B-cell malignancy, three had been treated with multiagent chemotherapy.
  • Two patients who presented with early-stage mycosis fungoides (IA) as the first lymphoma developed mycosis fungoides tumours after becoming immunosuppressed.
  • In two patients infiltrates composed of both malignant T- and B-cell populations were found in a single biopsy.
  • One showed two distinct populations of the malignant cells in the skin tumour, thus constituting a classical composite lymphoma of mycosis fungoides and chronic lymphocytic leukaemia, while in the other patient the two malignant populations of marginal B-cell lymphoma and mycosis fungoides (as evidenced by both phenotypic and genotypic findings) were intermingled.
  • CONCLUSIONS: This case series indicates that while the coexistence of Hodgkin's lymphoma and mycosis fungoides is extremely rare, the association of mycosis fungoides and B-cell malignancies is not as rare as reflected in the literature, with non-Hodgkin's lymphoma constituting the most common associated B-cell malignancy.
  • In this series as well as in the cases reported in the literature mycosis fungoides usually preceded the development of B-cell malignancies, which may be in accordance with previous reports of an increased risk of developing a second haematological neoplasm.
  • The importance of a competent immune system for patients with mycosis fungoides is well demonstrated in these cases.
  • It is suggested that for greater precision the criteria for diagnosis of composite lymphoma of the skin should include both phenotypic and genotypic features.
  • [MeSH-major] Lymphoma, B-Cell / pathology. Mycosis Fungoides / pathology. Neoplasms, Multiple Primary / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Hodgkin Disease / pathology. Humans. Leukemia, Lymphocytic, Chronic, B-Cell / pathology. Lymphoma, T-Cell, Peripheral / pathology. Male. Middle Aged

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  • (PMID = 16882178.001).
  • [ISSN] 0007-0963
  • [Journal-full-title] The British journal of dermatology
  • [ISO-abbreviation] Br. J. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] England
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12. Nikkels AF, Quatresooz P, Delvenne P, Balsat A, Piérard GE: Mycosis fungoides progression and chronic solvent exposure. Dermatology; 2004;208(2):171-3
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  • [Title] Mycosis fungoides progression and chronic solvent exposure.
  • The effect of repeated exposure to specific chemicals on the initiation or progression of mycosis fungoides (MF) remains unsettled.
  • A patient with low-grade patch stage MF progressively developed MF plaques restricted to his arms, and a tumour on his right thigh.
  • Immunophenotyping these lesions revealed a dense LCA+, CD2+, CD3+, CD4+, CD5+, CD7+, CD45+, CD45RO+ T-cell infiltrate admixed with many factor XIIIa+ dendrocytes.
  • T-cell receptor rearrangement analysis identified a monoclonal T-cell infiltrate.
  • This observation suggests that long-term solvent exposure may trigger MF and hasten its progression from the patch stage to the plaque and tumour stages.
  • [MeSH-major] Mycosis Fungoides / chemically induced. Mycosis Fungoides / pathology. Occupational Exposure / adverse effects. Skin Neoplasms / chemically induced. Skin Neoplasms / pathology. Solvents / adverse effects
  • [MeSH-minor] Adrenal Cortex Hormones / administration & dosage. Biopsy, Needle. Disease Progression. Drug Therapy, Combination. Follow-Up Studies. Humans. Immunohistochemistry. Interferon-alpha / administration & dosage. Male. Middle Aged. Paint / adverse effects. Patch Tests. Recombinant Proteins. Risk Assessment. Time Factors

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  • [Copyright] Copyright 2004 S. Karger AG, Basel
  • (PMID = 15057012.001).
  • [ISSN] 1018-8665
  • [Journal-full-title] Dermatology (Basel, Switzerland)
  • [ISO-abbreviation] Dermatology (Basel)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Interferon-alpha; 0 / Recombinant Proteins; 0 / Solvents; 76543-88-9 / interferon alfa-2a
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13. Peycheva E, Daskalov I, Tsoneva I: Electrochemotherapy of Mycosis fungoides by interferon-alpha. Bioelectrochemistry; 2007 May;70(2):283-6
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  • [Title] Electrochemotherapy of Mycosis fungoides by interferon-alpha.
  • Eight patients with 29 lesions of histologically verified 1st stage of Mycosis fungoides were successfully treated by electrochemotherapy with interferon-alpha.
  • Compared to the traditional monoimmunotherapy with interferon-alpha applied three times weekly for a total of 4 weeks, the electrochemotherapy was very efficient.
  • The expected mechanism involved in multiple cytotoxic action of interferon-alpha could be the local increased concentration in the tumour and prolongation of the time of its action after the application of pulses.
  • [MeSH-major] Electrochemotherapy / methods. Interferon-alpha / administration & dosage. Mycosis Fungoides / drug therapy. Mycosis Fungoides / pathology. Skin Neoplasms / drug therapy. Skin Neoplasms / pathology
  • [MeSH-minor] Angiogenesis Inhibitors / administration & dosage. Antineoplastic Agents / administration & dosage. Humans. Injections, Intralesional / methods. Treatment Outcome

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  • (PMID = 17150416.001).
  • [ISSN] 1567-5394
  • [Journal-full-title] Bioelectrochemistry (Amsterdam, Netherlands)
  • [ISO-abbreviation] Bioelectrochemistry
  • [Language] eng
  • [Publication-type] Controlled Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antineoplastic Agents; 0 / Interferon-alpha
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14. Wozniak MB, Tracey L, Ortiz-Romero PL, Montes S, Alvarez M, Fraga J, Fernández Herrera J, Vidal S, Rodriguez-Peralto JL, Piris MA, Villuendas Deceased R: Psoralen plus ultraviolet A +/- interferon-alpha treatment resistance in mycosis fungoides: the role of tumour microenvironment, nuclear transcription factor-kappaB and T-cell receptor pathways. Br J Dermatol; 2009 Jan;160(1):92-102
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  • [Title] Psoralen plus ultraviolet A +/- interferon-alpha treatment resistance in mycosis fungoides: the role of tumour microenvironment, nuclear transcription factor-kappaB and T-cell receptor pathways.
  • BACKGROUND: Interferon (IFN)-alpha is widely used in the treatment of mycosis fungoides (MF) and when used in combination with photochemotherapy (psoralen plus ultraviolet A, PUVA) both improved response and duration of complete remission have been reported.
  • However, in spite of encouraging results of the initial studies, currently there is no information available on specific prognostic factors enabling prediction of patients' resistance to PUVA +/- IFN-alpha treatment.
  • OBJECTIVES: To identify factors responsible for resistance to PUVA +/- IFN-alpha treatment in MF patients.
  • PATIENTS/METHODS: The gene expression profiling of pretreatment samples from 29 patients diagnosed as IA, IB or IIA stage of MF enrolled in a randomized PUVA vs. PUVA + IFN-alpha clinical trial was analysed using cDNA microarrays.
  • A Cox model (SAM) and gene set enrichment analysis (GSEA) were used for identification of genes and biologically significant pathways related to resistance to treatment.
  • RESULTS: Genes involved in NF-kappaB signalling, T-cell receptor (TCR) signalling, cytokine signalling and proliferation were differentially expressed between responders and nonresponders.
  • Interestingly, expression of markers representative of those pathways was found not only in the tumoral cells, but also in specific subpopulations of macrophages, dendritic cells and other non-neoplastic cell types constituting the tumour microenvironment, likely involved in the promotion of survival and proliferation of cutaneous T-cell lymphoma.
  • CONCLUSIONS: Gene expression changes in both the tumour and the tumour microenvironment are an important determinant of treatment outcome in early-stage MF patients.
  • Some proinflammatory factors such as NF-kappaB, inflammatory cytokines and their receptors in addition to TCR-associated molecules could be promising targets for MF treatment.
  • [MeSH-major] Interferon-alpha / therapeutic use. Mycosis Fungoides / genetics. NF-kappa B / metabolism. Receptors, Antigen, T-Cell / metabolism. Skin Neoplasms / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Combined Modality Therapy. Drug Resistance, Neoplasm / genetics. Female. Gene Expression Profiling. Humans. Immunohistochemistry. Male. Middle Aged. PUVA Therapy / methods. Signal Transduction / genetics. Treatment Outcome. Young Adult

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  • (PMID = 18945306.001).
  • [ISSN] 1365-2133
  • [Journal-full-title] The British journal of dermatology
  • [ISO-abbreviation] Br. J. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Interferon-alpha; 0 / NF-kappa B; 0 / Receptors, Antigen, T-Cell
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15. Child FJ, Mitchell TJ, Whittaker SJ, Scarisbrick JJ, Seed PT, Russell-Jones R: A randomized cross-over study to compare PUVA and extracorporeal photopheresis in the treatment of plaque stage (T2) mycosis fungoides. Clin Exp Dermatol; 2004 May;29(3):231-6
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  • [Title] A randomized cross-over study to compare PUVA and extracorporeal photopheresis in the treatment of plaque stage (T2) mycosis fungoides.
  • PUVA is a well-established and effective treatment for plaque stage mycosis fungoides (MF) but its use is limited on a long-term basis because of the risk of cutaneous carcinogenesis.
  • Therefore it is important to find alternative methods of treatment.
  • Extracorporeal photopheresis (ECP) is a form of photochemotherapy that involves exposure of white blood cells to UVA with psoralens and can be effective in Sézary syndrome and erythrodermic cutaneous T-cell lymphoma.
  • The aim of this study was to compare the efficacy of PUVA and ECP in the treatment of patients with T2 plaque stage (Stage 1B) MF who had a detectable peripheral blood T-cell clone.
  • Response was assessed by monthly skin scores and peripheral blood T-cell clonality.
  • Skin scores taken at the completion of each treatment arm in patients who completed the study were 113 units better (confidence interval, 42-184 units) following 3 months PUVA than 6 months ECP (P = 0.002).
  • Peripheral blood T-cell clones were detectable in all patients post-treatment.
  • This study indicates that ECP is not effective in the treatment of plaque stage (1B/T2) MF even in patients with molecular evidence of a peripheral blood T-cell clone.
  • Although PUVA was more effective than ECP, neither treatment modality cleared malignant T-cells from the peripheral blood.
  • [MeSH-major] Mycosis Fungoides / drug therapy. PUVA Therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Cross-Over Studies. Female. Humans. Male. Middle Aged. Photopheresis / adverse effects. Severity of Illness Index. Treatment Outcome

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  • (PMID = 15115499.001).
  • [ISSN] 0307-6938
  • [Journal-full-title] Clinical and experimental dermatology
  • [ISO-abbreviation] Clin. Exp. Dermatol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article; Randomized Controlled Trial
  • [Publication-country] England
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16. Zackheim HS, Kashani-Sabet M, McMillan A: Low-dose methotrexate to treat mycosis fungoides: a retrospective study in 69 patients. J Am Acad Dermatol; 2003 Nov;49(5):873-8
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  • [Title] Low-dose methotrexate to treat mycosis fungoides: a retrospective study in 69 patients.
  • BACKGROUND: Although low-dose methotrexate has been used to treat mycosis fungoides for many years, documentation is very limited.
  • OBJECTIVE: Our purpose was to review our experience with methotrexate in the treatment of 69 patients with patch/plaque and tumor stage mycosis fungoides observed for up to 201 months.
  • Data are presented in terms of response rates and time to treatment failure.
  • RESULTS: The greatest number of patients (60) had patch/plaque stage T2 disease (>/=10% skin involved).
  • The median time to treatment failure was 15 months.
  • Only 1 of 7 patients with tumor stage disease responded.
  • Side effects caused treatment failure in 6 (9%) of the total cohort of 69 patients.
  • CONCLUSION: Low-dose methotrexate may be of value in the treatment of a subset of patients with patch/plaque mycosis fungoides resistant to other therapies.
  • [MeSH-major] Antimetabolites, Antineoplastic / administration & dosage. Methotrexate / administration & dosage. Mycosis Fungoides / drug therapy. Skin Neoplasms / drug therapy

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  • (PMID = 14576667.001).
  • [ISSN] 0190-9622
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; YL5FZ2Y5U1 / Methotrexate
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17. Lansigan F, Foss FM: Current and emerging treatment strategies for cutaneous T-cell lymphoma. Drugs; 2010 Feb 12;70(3):273-86
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  • [Title] Current and emerging treatment strategies for cutaneous T-cell lymphoma.
  • Cutaneous T-cell lymphomas (CTCLs) are a rare group of mature T-cell lymphomas presenting primarily in the skin.
  • The most common subtypes of CTCL are mycosis fungoides and its leukaemic variant Sézary's syndrome.
  • Patients with early-stage disease frequently have an indolent clinical course; however, those with advanced stages have a shortened survival.
  • For the treating physician, the question of how to choose a particular therapy in the management of CTCL is important.
  • Other than an allogeneic stem cell transplant, there are no curative therapies for this disease.
  • Hence, many treatment modalities need to be offered to the patient over the course of their life.
  • An accepted treatment approach has been to delay traditional chemotherapy, which can cause excessive toxicity without durable benefit.
  • More conservative treatment strategies in the initial management of CTCL have led to the development of newer biological and targeted therapies.
  • These therapies include biological immune enhancers such as interferon alpha and extracorporeal photopheresis that exert their effect by stimulating an immune response to the tumour cells.
  • Lastly, systemic chemotherapy including transplantation is used when rapid disease control is needed or if all other biological therapies have failed.
  • As response rates to most of the biological agents used to treat CTCL are 25-30%, it is also reasonable to consider clinical trials with novel agents if one or two front-line therapies have failed, especially before considering chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy / methods. Lymphoma, T-Cell, Cutaneous / drug therapy. Lymphoma, T-Cell, Cutaneous / therapy. Skin Neoplasms / drug therapy. Skin Neoplasms / therapy
  • [MeSH-minor] Antibodies, Monoclonal / therapeutic use. Clinical Protocols. Drug Administration Routes. Hematopoietic Stem Cell Transplantation / methods. Humans

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  • (PMID = 20166766.001).
  • [ISSN] 1179-1950
  • [Journal-full-title] Drugs
  • [ISO-abbreviation] Drugs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] New Zealand
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal
  • [Number-of-references] 90
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18. 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.
  • [Title] Staging and management of cutaneous T-cell lymphoma.
  • Cutaneous T-cell lymphoma (CTCL) accounts for two-thirds of cases of primary cutaneous lymphoma.
  • Most variants of CTCL are indolent lymphoma, the most common being mycosis fungoides.
  • Accurate diagnosis and staging is critical in determining the prognosis of those with CTCL.
  • The tumour, node, metastasis and blood stage needs to be documented and used to determine an overall stage from IA to IVB.
  • Thorough investigations are needed at diagnosis and should be repeated during disease progression to allow initial staging and restaging.
  • 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-major] Lymphoma, T-Cell, Cutaneous / pathology. Neoplasm Staging / methods. Skin Neoplasms
  • [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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19. 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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  • [Title] Long-term remission after allogeneic hematopoietic stem cell transplantation for refractory cutaneous T-cell lymphoma.
  • 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.
  • One patient was in complete remission for 9 months, followed by limited cutaneous recurrence.
  • Mild graft-vs-host disease and graft-vs-tumor effect have contained the recurring disease as a low-grade process.
  • CONCLUSIONS: Allogeneic hematopoietic stem cell transplantation has the potential for sustained remission and the possibility of cure for young patients with advanced and recalcitrant cutaneous T-cell lymphoma.
  • Even in the absence of complete remission, an allogeneic graft-vs-tumor effect may provide an immune mechanism to control the malignant T-cell process and alter the natural history of disease.
  • [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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20. Amitay-Laish I, David M, Hodak E: Systemic progression following complete cutaneous remission under bexarotene treatment for tumor-stage mycosis fungoides. J Am Acad Dermatol; 2009 Aug;61(2):361-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Systemic progression following complete cutaneous remission under bexarotene treatment for tumor-stage mycosis fungoides.
  • [MeSH-major] Mycosis Fungoides / therapy. Neoplasm Invasiveness / pathology. Neoplasm Recurrence, Local / drug therapy. Skin Neoplasms / therapy. Tetrahydronaphthalenes / therapeutic use
  • [MeSH-minor] Biopsy, Needle. Combined Modality Therapy. Disease Progression. Dose-Response Relationship, Drug. Drug Administration Schedule. Follow-Up Studies. Humans. Immunohistochemistry. Male. Middle Aged. Retreatment. Risk Assessment. Treatment Outcome

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  • [CommentOn] J Am Acad Dermatol. 2005 Jun;52(6):991-6 [15928617.001]
  • (PMID = 19615550.001).
  • [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; Comment; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Tetrahydronaphthalenes; A61RXM4375 / bexarotene
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21. Wobser M, Göppner D, Lang SC, Beckmann G, Flentje M, Ugurel S, Bröcker EB, Becker JC: Durable complete remission of therapy-refractory, tumor-stage cutaneous T-cell lymphoma under radioimmunotherapy with electron beam irradiation and denileukin diftitox. Arch Dermatol; 2010 Jul;146(7):805-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Durable complete remission of therapy-refractory, tumor-stage cutaneous T-cell lymphoma under radioimmunotherapy with electron beam irradiation and denileukin diftitox.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Diphtheria Toxin / therapeutic use. Interleukin-2 / therapeutic use. Lymphoma, T-Cell, Cutaneous / therapy. Remission Induction / methods. Skin Neoplasms / therapy
  • [MeSH-minor] Aged, 80 and over. Dose-Response Relationship, Drug. Follow-Up Studies. Foot. Humans. Male. Neoplasm Staging. Radiotherapy, Adjuvant. Recombinant Fusion Proteins / administration & dosage. Recombinant Fusion Proteins / therapeutic use

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  • (PMID = 20644054.001).
  • [ISSN] 1538-3652
  • [Journal-full-title] Archives of dermatology
  • [ISO-abbreviation] Arch Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Diphtheria Toxin; 0 / Interleukin-2; 0 / Recombinant Fusion Proteins; 25E79B5CTM / denileukin diftitox
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