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1. Duvic M, Foss FM: Mycosis fungoides: pathophysiology and emerging therapies. Semin Oncol; 2007 Dec;34(6 Suppl 5):S21-8
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  • [Title] Mycosis fungoides: pathophysiology and emerging therapies.
  • Mycosis fungoides and its leukemic variant Sézary syndrome represent the most common CTCL subtypes.
  • Current treatment for patients with mycosis fungoides involves topical and systemic therapies for the cutaneous manifestations.
  • However, no therapy is curative and patients often progress to advanced extracutaneous CTCL with visceral organ complications or relapsed disease that is frequently refractory to most topical and aggressive systemic regimens.
  • The emergence of novel targeted therapies such as biologic agents, histone deacetylase inhibitors, and purine nucleoside phosphorylase inhibitors offers promise for more effective and safer treatment strategies for refractory CTCLs.
  • [MeSH-major] Histone Deacetylases / drug effects. Mycosis Fungoides. Purine-Nucleoside Phosphorylase / drug effects. Skin Neoplasms
  • [MeSH-minor] Clinical Trials as Topic. Humans. Immunologic Factors / therapeutic use. Purine Nucleosides / therapeutic use. Pyrimidinones / therapeutic use. Sezary Syndrome / drug therapy. Sezary Syndrome / physiopathology

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  • (PMID = 18086343.001).
  • [ISSN] 0093-7754
  • [Journal-full-title] Seminars in oncology
  • [ISO-abbreviation] Semin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunologic Factors; 0 / Purine Nucleosides; 0 / Pyrimidinones; 426X066ELK / forodesine; EC 2.4.2.1 / Purine-Nucleoside Phosphorylase; EC 3.5.1.98 / Histone Deacetylases
  • [Number-of-references] 56
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2. Zinzani PL, Baliva G, Magagnoli M, Bendandi M, Modugno G, Gherlinzoni F, Orcioni GF, Ascani S, Simoni R, Pileri SA, Tura S: Gemcitabine treatment in pretreated cutaneous T-cell lymphoma: experience in 44 patients. J Clin Oncol; 2000 Jul;18(13):2603-6
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  • [Title] Gemcitabine treatment in pretreated cutaneous T-cell lymphoma: experience in 44 patients.
  • PURPOSE: To evaluate the efficacy and toxicity of gemcitabine, a novel pyrimidine antimetabolite with a low-toxicity profile and activity in several solid tumors, in patients with relapsed or refractory cutaneous T-cell lymphomas.
  • PATIENTS AND METHODS: Between May 1997 and February 1999, 44 previously treated patients with mycosis fungoides (MF; n = 30) and peripheral T-cell lymphoma unspecified (PTCLU) with exclusive skin involvement (n = 14) were enrolled onto a two-institution, phase II trial and treated with gemcitabine.
  • This drug was given on days 1, 8, and 15 of a 28-day schedule at a dose of 1,200 mg/m(2) intravenously over 30 minutes for a total of three courses.
  • 5%) achieved complete responses (CRs), 26 (59%) partial responses (PRs), and the remaining 13 showed no benefit from the treatment.
  • No difference in terms of overall response rate was observed between relapsed and refractory patients.
  • Treatment was well tolerated; hematologic toxicity was mild, and no nausea/vomiting or organ toxicity was recorded.
  • Further studies that use gemcitabine alone or in combination with other drugs in earlier stages of the disease are needed.
  • [MeSH-major] Antimetabolites, Antineoplastic / therapeutic use. Deoxycytidine / analogs & derivatives. Lymphoma, T-Cell, Cutaneous / drug therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Humans. Male. Middle Aged. Mycosis Fungoides / drug therapy. Mycosis Fungoides / mortality. Mycosis Fungoides / pathology. Survival Rate

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  • (PMID = 10893292.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0W860991D6 / Deoxycytidine; B76N6SBZ8R / gemcitabine
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3. Zinzani PL, Venturini F, Stefoni V, Fina M, Pellegrini C, Derenzini E, Gandolfi L, Broccoli A, Argnani L, Quirini F, Pileri S, Baccarani M: Gemcitabine as single agent in pretreated T-cell lymphoma patients: evaluation of the long-term outcome. Ann Oncol; 2010 Apr;21(4):860-3
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  • BACKGROUND: Peripheral T-cell lymphoma unspecified (PTCLU) and mycosis fungoides (MF) often show resistance to conventional chemotherapy.
  • Inclusion criteria were as follows: histologic diagnosis of MF or PTCLU; relapsed/refractory disease; age > or =18 years; and World Health Organization performance status of two or less.

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  • (PMID = 19887465.001).
  • [ISSN] 1569-8041
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0W860991D6 / Deoxycytidine; B76N6SBZ8R / gemcitabine
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4. Ogura M, Morishima Y, Kobayashi Y, Uike N, Sugai S, Chou T, Kasai M, Miura I, Murayama T, Matsuno Y, Nakamura S, Mori S, Ohashi Y, Tobinai K, Cladribine Study Group: Durable response but prolonged cytopenia after cladribine treatment in relapsed patients with indolent non-Hodgkin's lymphomas: results of a Japanese phase II study. Int J Hematol; 2004 Oct;80(3):267-77
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  • [Title] Durable response but prolonged cytopenia after cladribine treatment in relapsed patients with indolent non-Hodgkin's lymphomas: results of a Japanese phase II study.
  • We conducted a phase II study to evaluate the efficacy and safety of cladribine (2-chlorodeoxyadenosine [2-CdA]) for patients with refractory or relapsed indolent B-cell lymphoma or mycosis fungoides.
  • Neutropenia and thrombocytopenia of grade 3 or 4 were observed in 53.3% and 37.8% of patients, respectively, with prolonged cytopenia observed in patients with increased numbers of treatment cycles.
  • Two treatment-related deaths were observed.
  • Four patients developed myelodysplastic syndrome (MDS) at 13 months to 2 years after completion of the 2-CdA treatments.
  • 2-CdA is an active agent with acceptable toxicity for refractory or relapsed indolent lymphoma; however, prolonged myelosuppression and the potential development of MDS should be carefully monitored.
  • [MeSH-major] Cladribine / administration & dosage. Cladribine / toxicity. Lymphoma, Non-Hodgkin / drug therapy. Pancytopenia / chemically induced
  • [MeSH-minor] Adult. Aged. Female. Humans. Japan. Lymphoma, B-Cell / complications. Lymphoma, B-Cell / drug therapy. Male. Middle Aged. Mycosis Fungoides / complications. Mycosis Fungoides / drug therapy. Myelodysplastic Syndromes / chemically induced. Neoplasms, Second Primary / chemically induced. Recurrence. Remission Induction / methods. Salvage Therapy / methods. Survival Analysis

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  • (PMID = 15540903.001).
  • [ISSN] 0925-5710
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 47M74X9YT5 / Cladribine
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5. Querfeld C, Rosen ST, Guitart J, Rademaker A, Fung BB, Posten W, Kuzel TM: Comparison of selective retinoic acid receptor- and retinoic X receptor-mediated efficacy, tolerance, and survival in cutaneous t-cell lymphoma. J Am Acad Dermatol; 2004 Jul;51(1):25-32
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The most common subtypes of cutaneous T-cell lymphomas are the epidermotropic variants mycosis fungoides and Sézary syndrome.
  • Treatment of mycosis fungoides has encompassed a variety of modalities including the use of retinoids with several studies evaluating their efficacy.
  • The objective of our retrospective, nonrandomized, single-center study was to compare the response, survival outcomes, and toxic effects in our phase II trial of the RAR-specific retinoid, all-trans retinoic acid, with clinical use of the RXR-specific retinoid, bexarotene, in patients with mycosis fungoides/Sézary syndrome who have relapsed.
  • There was no statistical difference in response rates (12% vs 21%), response duration (20.5 vs 7.3 months), event-free survival time (4 vs 5 months), or median survival when corrected for length of follow-up.
  • Both have favorable toxicity profiles that can be managed with medications.
  • However, the immunomodulatory effects of RAR and RXR retinoids provide a rational basis for using retinoids in combination with other biologic immune response modifiers, phototherapy, or cytotoxic chemotherapy.
  • [MeSH-major] Anticarcinogenic Agents / therapeutic use. Antineoplastic Agents / therapeutic use. Lymphoma, T-Cell, Cutaneous / drug therapy. Tetrahydronaphthalenes / therapeutic use. Tretinoin / therapeutic use
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Follow-Up Studies. Humans. Male. Middle Aged. Receptors, Retinoic Acid. Retinoid X Receptors. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 15243520.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anticarcinogenic Agents; 0 / Antineoplastic Agents; 0 / Receptors, Retinoic Acid; 0 / Retinoid X Receptors; 0 / Tetrahydronaphthalenes; 5688UTC01R / Tretinoin; A61RXM4375 / bexarotene
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6. 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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  • 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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7. Du-Thanh A, Durand L, Costes V, Guillot B, Dereure O: [Aggressive T cytotoxic CD8+ epidermotropic cutaneous lymphoma: a case in a patient with Steinert's myotonic dystrophy]. Ann Dermatol Venereol; 2006 Dec;133(12):991-4
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  • [Transliterated title] Lymphome T cutané épidermotrope cytotoxique CD8+ "agressif": difficultés de prise en charge chez un malade atteint d'une dystrophie myotonique de Steinert.
  • INTRODUCTION: Primary cutaneous "aggressive" CD8-positive epidermotropic cytotoxic T-cell lymphoma is a rare subset of cutaneous cytotoxic T/NK lymphomas that clearly differs from mycosis fungoides, whether CD4+ or CD8+, by the presence of rapidly evolving tumoral cutaneous lesions, foci of keratinocytes necrosis, a cytotoxic T phenotype and a poor prognosis.
  • CHOP chemotherapy was effective despite cardiac toxicity in the setting of Steinert's dystrophy, but the patient relapsed and died of pulmonary sepsis after chemotherapy was resumed.
  • DISCUSSION: The treatment of primary cutaneous epidermotropic CD8+ cytotoxic T-cell lymphoma is not codified.
  • CHOP chemotherapy is usually the first-line therapy but relapses are frequent with median survival of no more than 34 months.
  • In our patient, an additional difficulty was the cardiac toxicity of cytostatic drugs linked to the myopathy which prevented the use of high dosages, requiring a change of therapeutic regimen.
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclophosphamide / therapeutic use. Doxorubicin / therapeutic use. Humans. Male. Prednisone / therapeutic use. Vincristine / therapeutic use

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  • (PMID = 17185931.001).
  • [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
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone; CHOP protocol
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8. Olsen EA, Kim YH, Kuzel TM, Pacheco TR, Foss FM, Parker S, Frankel SR, Chen C, Ricker JL, Arduino JM, Duvic M: Phase IIb multicenter trial of vorinostat in patients with persistent, progressive, or treatment refractory cutaneous T-cell lymphoma. J Clin Oncol; 2007 Jul 20;25(21):3109-15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phase IIb multicenter trial of vorinostat in patients with persistent, progressive, or treatment refractory cutaneous T-cell lymphoma.
  • PURPOSE: To evaluate the activity and safety of the histone deacetylase inhibitor vorinostat (suberoylanilide hydroxamic acid) in persistent, progressive, or recurrent mycosis fungoides or Sézary syndrome (MF/SS) cutaneous t-cell lymphoma (CTCL) subtypes.
  • Patients must have received at least two prior systemic therapies at least one of which included bexarotene unless intolerable.
  • The primary end point was the objective response rate (ORR) measured by the modified severity weighted assessment tool and secondary end points were time to response (TTR), time to progression (TTP), duration of response (DOR), and pruritus relief ( > or = 3-point improvement on a 10-point visual analog scale).
  • The most common drug-related adverse experiences (AE) were diarrhea (49%), fatigue (46%), nausea (43%), and anorexia (26%); most were grade 2 or lower but those grade 3 or higher included fatigue (5%), pulmonary embolism (5%), thrombocytopenia (5%), and nausea (4%).
  • CONCLUSION: Oral vorinostat was effective in treatment refractory MF/SS with an acceptable safety profile.
  • [MeSH-major] Hydroxamic Acids / therapeutic use. Lymphoma, T-Cell, Cutaneous / drug therapy. Salvage Therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Administration, Oral. Adult. Aged. Aged, 80 and over. Confidence Intervals. Dose-Response Relationship, Drug. Drug Administration Schedule. Drug Resistance, Neoplasm. Female. Follow-Up Studies. Humans. Male. Maximum Tolerated Dose. Middle Aged. Neoplasm Staging. Probability. Survival Analysis. Treatment Outcome


9. Querfeld C, Mehta N, Rosen ST, Guitart J, Rademaker A, Gerami P, Kuzel TM: Alemtuzumab for relapsed and refractory erythrodermic cutaneous T-cell lymphoma: a single institution experience from the Robert H. Lurie Comprehensive Cancer Center. Leuk Lymphoma; 2009 Dec;50(12):1969-76
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alemtuzumab for relapsed and refractory erythrodermic cutaneous T-cell lymphoma: a single institution experience from the Robert H. Lurie Comprehensive Cancer Center.
  • We present the results of an open-label clinical trial and the clinical use of alemtuzumab in 19 heavily pretreated patients with advanced erythrodermic cutaneous T-cell lymphomas (CTCL) (erythrodermic mycosis fungoides and Sézary syndrome).
  • Ten patients received alemtuzumab intravenously using an escalating dose regimen with a final dose of 30 mg three times weekly for 4 weeks followed by subcutaneous administration for 8 weeks.
  • One patient was diagnosed with a concurrent lymphoma (mantle cell lymphoma) 6 months after completing alemtuzumab therapy.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antibodies, Neoplasm / therapeutic use. Mycosis Fungoides / drug therapy. Sezary Syndrome / drug therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Aged. Aged, 80 and over. Antibodies, Monoclonal, Humanized. Antigens, CD / analysis. Antigens, Neoplasm / analysis. Antineoplastic Agents / adverse effects. Antineoplastic Agents / therapeutic use. Dose-Response Relationship, Drug. Drug Administration Schedule. Drug Resistance, Neoplasm. Fatigue / chemically induced. Female. Flow Cytometry. Glycoproteins / analysis. Humans. Leukopenia / chemically induced. Male. Middle Aged. Neoplasm, Residual / diagnosis. Neoplasm, Residual / drug therapy. Pruritus / chemically induced. Remission Induction. Survival Analysis. T-Lymphocytes / metabolism. T-Lymphocytes / pathology. Treatment Outcome

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  • (PMID = 19860617.001).
  • [ISSN] 1029-2403
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [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 / Glycoproteins; 3A189DH42V / alemtuzumab
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10. Ishida M, Hotta M, Takikita-Suzuki M, Kojima F, Okabe H: CD8-positive granulomatous mycosis fungoides: a case report with review of the literature. J Cutan Pathol; 2010 Oct;37(10):1072-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] CD8-positive granulomatous mycosis fungoides: a case report with review of the literature.
  • Granulomatous mycosis fungoides (GMF) represents an uncommon variant of mycosis fungoides (MF) characterized by the presence of an associated granulomatous reaction.
  • She had been diagnosed with MF, and most of the eruption improved by psoralen ultraviolet A therapy.
  • However, the eruption relapsed and gradually expanded 5 months prior to her visit to our hospital.
  • [MeSH-major] Antigens, CD8 / biosynthesis. Granuloma / pathology. Mycosis Fungoides / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Aged. Diabetes Mellitus / pathology. Female. Humans. Immunohistochemistry. Neoplasm Recurrence, Local / drug therapy. Neoplasm Recurrence, Local / metabolism. Neoplasm Recurrence, Local / pathology. PUVA Therapy

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  • (PMID = 20579213.001).
  • [ISSN] 1600-0560
  • [Journal-full-title] Journal of cutaneous pathology
  • [ISO-abbreviation] J. Cutan. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antigens, CD8
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11. Bittencourt AL, Mota K, Oliveira RF, Farré L: A dyshidrosis-like variant of adult T-cell leukemia/lymphoma with clinicopathological aspects of mycosis fungoides. A case report. Am J Dermatopathol; 2009 Dec;31(8):834-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A dyshidrosis-like variant of adult T-cell leukemia/lymphoma with clinicopathological aspects of mycosis fungoides. A case report.
  • Adult T-cell leukemia/lymphoma (ATL) is an aggressive type of leukemia/lymphoma associated with the human T-cell lymphotropic virus (HTLV-I).
  • We describe an adult male patient clinically and pathologically diagnosed as mycosis fungoides and treated with chemotherapy after which complete involution of the lesions occurred.
  • The disease relapsed with confluent dyshidrosis-like vesicles on the palmoplantar regions, followed by disseminated vesiculopapules and associated lymphocytosis.
  • A serological test performed at this time revealed HTLV-I infection, and a diagnosis of chronic ATL was made.
  • The delay that occurred in diagnosing ATL was due to the fact that mycosis fungoides and ATL may present the same clinical, histopathological, and immunohistochemical features.
  • [MeSH-major] Eczema, Dyshidrotic / pathology. HTLV-I Infections / pathology. Leukemia-Lymphoma, Adult T-Cell / pathology. Mycosis Fungoides / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclophosphamide. Diabetes Mellitus. Diagnosis, Differential. Doxorubicin. Human T-lymphotropic virus 1. Humans. Immunohistochemistry. Interferon-alpha / therapeutic use. Male. Polymerase Chain Reaction. Prednisone. Vincristine. Zidovudine / therapeutic use

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  • (PMID = 19770630.001).
  • [ISSN] 1533-0311
  • [Journal-full-title] The American Journal of dermatopathology
  • [ISO-abbreviation] Am J Dermatopathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Interferon-alpha; 4B9XT59T7S / Zidovudine; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone; CHOP protocol
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12. Papadavid E, Antoniou C, Nikolaou V, Siakantaris M, Vassilakopoulos TP, Stratigos A, Stavrianeas N, Katsambas A: Safety and efficacy of low-dose bexarotene and PUVA in the treatment of patients with mycosis fungoides. Am J Clin Dermatol; 2008;9(3):169-73
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Safety and efficacy of low-dose bexarotene and PUVA in the treatment of patients with mycosis fungoides.
  • Although combinations of oral bexarotene and psoralen plus UVA (PUVA) have been tried in patients with all stages of mycosis fungoides (MF), the dosage of bexarotene used in these combination regimens has been variable.
  • OBJECTIVE: To assess the efficacy and safety of low-dose oral bexarotene and PUVA in patients with relapsed or treatment-refractory MF following monotherapy with multiple agents including PUVA, narrow-band UVB, interferon-alpha, oral bexarotene, and topical corticosteroids.
  • METHOD: Combination therapy with PUVA three times weekly and low-dose oral bexarotene (150 or 300 mg/day, depending on physicians' preference) was administered to 14 patients, seven men and seven women (median age 49.5 years, range 30-75 years), with relapsed or refractory MF stages I-III.
  • All responders received maintenance treatment at the same bexarotene dose that induced remission until progression or unacceptable toxicity.
  • RESULTS: Low-dose oral bexarotene combined with PUVA was associated with an overall response rate (complete response or partial response) in 67% of the nine patients with refractory MF who completed the treatment course.
  • Oral bexarotene was continued as maintenance therapy in three of the four complete responders (one refused); two of these patients relapsed 2-10 months after PUVA discontinuation.
  • Patients with partial response or stable disease received the combination for 3-5 months and were switched to another treatment regimen because of lack of further response.
  • Therapy was fairly well tolerated.
  • CONCLUSION: In a select population of patients who had not responded to at least one monotherapy for early-stage MF, a combination of low-dose oral bexarotene and PUVA was successful in achieving a satisfactory overall response rate in 67% of patients who completed the treatment course and was fairly well tolerated.
  • [MeSH-major] Mycosis Fungoides / drug therapy. Tetrahydronaphthalenes / therapeutic use
  • [MeSH-minor] Adult. Aged. Anticarcinogenic Agents / adverse effects. Anticarcinogenic Agents / therapeutic use. Drug Therapy, Combination. Female. Humans. Male. Middle Aged. PUVA Therapy. Treatment Outcome

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  • (PMID = 18429646.001).
  • [ISSN] 1175-0561
  • [Journal-full-title] American journal of clinical dermatology
  • [ISO-abbreviation] Am J Clin Dermatol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] New Zealand
  • [Chemical-registry-number] 0 / Anticarcinogenic Agents; 0 / Tetrahydronaphthalenes; A61RXM4375 / bexarotene
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13. Huber MA, Staib G, Pehamberger H, Scharffetter-Kochanek K: Management of refractory early-stage cutaneous T-cell lymphoma. Am J Clin Dermatol; 2006;7(3):155-69
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Mycosis fungoides is recognized as the most common type of CTCL.
  • However, although there is a wide array of therapeutic options for early-stage CTCL, not all patients respond to these individual therapies, resulting in refractory cutaneous disease over time.
  • Refractory early-stage CTCL poses an important therapeutic challenge, as one of the principal treatment goals is to keep the disease confined to the skin, thereby preventing disease progression.
  • Much of the focus of current research has been on the evaluation of already available skin-directed therapies and biologic response modifiers and combination regimens thereof, such as the combination of psoralen and UVA (PUVA) with interferon-alpha or retinoids.
  • Likewise, the topical gel formulation of bexarotene has proved to be an important therapeutic option in patients with refractory or relapsed lesions.
  • Oral bexarotene and topical bexarotene have been approved by the US FDA for the treatment of refractory CTCL.
  • Systemic chemotherapy is typically reserved for advanced-stage CTCL and is usually not recommended for early-stage, skin-limited disease.
  • However, recent exploratory studies indicate that low-dose methotrexate may represent an overall well tolerated therapy in a subset of patients with refractory early-stage CTCL, as may pegylated liposomal doxorubicin, which is currently being investigated in this specific clinical setting.
  • Another recently FDA-approved therapy is the interleukin-2 fusion toxin denileukin diftitox, which is now well established to play a role in the treatment of refractory CTCL, including early-stage extensive plaque disease.
  • [MeSH-major] Lymphoma, T-Cell, Cutaneous / therapy. Skin Neoplasms / therapy
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Humans. Immunologic Factors / therapeutic use. Phototherapy / methods. Retinoids / therapeutic use

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  • (PMID = 16734503.001).
  • [ISSN] 1175-0561
  • [Journal-full-title] American journal of clinical dermatology
  • [ISO-abbreviation] Am J Clin Dermatol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] New Zealand
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Antineoplastic Agents; 0 / Immunologic Factors; 0 / Retinoids
  • [Number-of-references] 93
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14. Gribben JG, Hallek M: Rediscovering alemtuzumab: current and emerging therapeutic roles. Br J Haematol; 2009 Mar;144(6):818-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rediscovering alemtuzumab: current and emerging therapeutic roles.
  • The humanized anti-CD52 monoclonal antibody alemtuzumab belongs to the family of Campath-1 antibodies, which were initially developed for their ability to prevent graft-versus-host disease (GVHD) and graft rejection in stem cell transplantation.
  • Alemtuzumab is indicated for the treatment of chronic lymphocytic leukaemia (CLL) and has demonstrated considerable activity in relapsed/refractory disease and in previously untreated disease.
  • It has been shown to induce minimal residual disease-negative responses as a single agent or as part of consolidation therapy in a meaningful proportion of patients with CLL and has shown promising activity in patients with high-risk cytogenetic markers.
  • Alemtuzumab may also have significant activity in T-cell malignancies, such as mycosis fungoides and T-cell prolymphocytic leukaemia.
  • This article reviews our current understanding of alemtuzumab and discusses its emerging role in the treatment of CLL and other haematological malignancies.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antibodies, Neoplasm / therapeutic use. Antineoplastic Agents / therapeutic use
  • [MeSH-minor] Antibodies, Monoclonal, Humanized. Antigens, CD / immunology. Antigens, Neoplasm / immunology. Glycoproteins / immunology. Graft vs Host Disease / prevention & control. Humans. Leukemia, Lymphocytic, Chronic, B-Cell / drug therapy. Stem Cell Transplantation. Transplantation Conditioning

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  • (PMID = 19183194.001).
  • [ISSN] 1365-2141
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P01 CA081534
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [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 / Glycoproteins; 3A189DH42V / alemtuzumab
  • [Number-of-references] 80
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15. 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.
  • 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


16. Talpur R, Apisarnthanarax N, Ward S, Duvic M: Treatment of refractory peripheral T-cell lymphoma with denileukin diftitox (ONTAK). Leuk Lymphoma; 2002 Jan;43(1):121-6
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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 refractory peripheral T-cell lymphoma with denileukin diftitox (ONTAK).
  • In general, their prognosis is poor, and specific therapy is not well defined.
  • We report the successful treatment of a patient with relapsed, refractory PTCL who after failing 13 standard single and multiple chemotherapy regimens and experimental agents had a dramatic prolonged response to diftitoxin denileukin (ONTAK).
  • This fusion protein, composed of diphtheria toxin coupled to interleukin-2, is approved for cutaneous T-cell lymphomas, including mycosis fungoides, and should be considered for treatment of the rare subset of peripheral T-cell lymphomas.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Diphtheria Toxin / therapeutic use. Interleukin-2 / therapeutic use. Lymphoma, T-Cell, Peripheral / drug therapy. Recombinant Fusion Proteins / therapeutic use
  • [MeSH-minor] Aged. Erythema / etiology. Humans. Lung Neoplasms / drug therapy. Lung Neoplasms / pathology. Lymph Nodes / pathology. Male. Nose Neoplasms / drug therapy. Nose Neoplasms / pathology. Receptors, Interleukin-2 / drug effects. Receptors, Interleukin-2 / metabolism. Salvage Therapy. Skin Neoplasms / drug therapy. Skin Neoplasms / pathology. Treatment Outcome

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  • (PMID = 11908715.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R21-CA74117
  • [Publication-type] Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Diphtheria Toxin; 0 / Interleukin-2; 0 / Receptors, Interleukin-2; 0 / Recombinant Fusion Proteins; 25E79B5CTM / denileukin diftitox
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17. Tsuji H, Wada T, Murakami M, Kashiwagi T, Ito Y, Ishida-Yamamoto A, Jimbo J, Shindo M, Sato K, Kohgo Y, Iizuka H: Two cases of mycosis fungoides treated by reduced-intensity cord blood transplantation. J Dermatol; 2010 Dec;37(12):1040-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Two cases of mycosis fungoides treated by reduced-intensity cord blood transplantation.
  • Mycosis fungoides is a cutaneous T-cell lymphoma, which is clinically divided into three stages: patch, plaque and tumor.
  • Despite a variety of treatments the prognosis is poor in advanced mycosis fungoides.
  • We performed reduced-intensity umbilical cord blood transplantation for two advanced mycosis fungoides patients.
  • Tumors of each case were refractory to conventional chemotherapy.
  • Although radiation therapy was considerably effective, tumors relapsed after several months.
  • However, chemotherapy-resistant tumors relapsed, and allogeneic hematopoietic stem cell transplantation was performed at 17 months.
  • She died of cerebral hemorrhage 23 days after the procedure.
  • Reduced-intensity umbilical cord blood transplantation may be included in the treatments for advanced mycosis fungoides, where graft-versus-lymphoma effect seems to be a significant factor for the success of the treatment.
  • [MeSH-major] Cord Blood Stem Cell Transplantation / methods. Mycosis Fungoides / surgery. Neoplasm Recurrence, Local / surgery. Skin Neoplasms / surgery
  • [MeSH-minor] Adult. Fatal Outcome. Female. Humans. Male. Middle Aged. Treatment Outcome. Young Adult

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  • [Copyright] © 2010 Japanese Dermatological Association.
  • (PMID = 21083707.001).
  • [ISSN] 1346-8138
  • [Journal-full-title] The Journal of dermatology
  • [ISO-abbreviation] J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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