Sezary Syndrome (Sezary Erythroderma)

A form of cutaneous T-cell lymphoma manifested by generalized exfoliative ERYTHRODERMA; PRURITUS; peripheral lymphadenopathy, and abnormal hyperchromatic mononuclear (cerebriform) cells in the skin, LYMPH NODES, and peripheral blood (Sezary cells).

Also Known As:

Networked: 123 relevant articles (10 outcomes, 12 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Mycosis Fungoides
2.	Cutaneous T-Cell Lymphoma (Lymphoma, T Cell, Cutaneous)
3.	B-Cell Chronic Lymphocytic Leukemia (Chronic Lymphocytic Leukemia)
4.	Graft vs Host Disease (Graft-Versus-Host Disease)
5.	Autoimmune Diseases (Autoimmune Disease)

Experts

1.	Rook, Alain H: 6 articles (02/2012 - 06/2003)
2.	Wysocka, Maria: 4 articles (02/2012 - 06/2003)
3.	Morrissey, Kelly A: 3 articles (02/2012 - 01/2010)
4.	Benoit, Bernice M: 3 articles (02/2012 - 05/2007)
5.	Duvic, Madeleine: 3 articles (05/2010 - 01/2002)
6.	Vonderheid, Eric C: 3 articles (06/2008 - 01/2002)
7.	Cutler, C S: 2 articles (11/2014 - 07/2011)
8.	Kim, Ellen J: 2 articles (02/2012 - 12/2011)
9.	Showe, Louise C: 2 articles (02/2012 - 06/2003)
10.	Vittorio, Carmela C: 2 articles (02/2012 - 12/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sezary Syndrome:

1.	vorinostat (suberoylanilide hydroxamic acid)FDA Link 09/01/2007 - "In two phase II trials, Vorinostat was safe and effective at an oral dose of 400 mg/day with an overall response rate of 30-31% in refractory advanced patients with CTCL including large cell transformation and Sezary syndrome. " 02/01/2012 - "We describe a patient with Sezary syndrome who was monitored for drug-induced immunosuppression while undergoing treatment with vorinostat. " 04/01/2011 - "We aim to evaluate the activity of vorinostat in combination with interferon (IFN) alpha and extracorporeal photopheresis (ECP) with persistent, progressive advanced stage MF and Sezary syndrome (SS). " 04/01/2011 - "The efficacy of vorinostat in combination with interferon alpha and extracorporeal photopheresis in late stage mycosis fungoides and Sezary syndrome."
2.	Complementarity Determining Regions (Complementarity Determining Region)IBA 08/01/2010 - "In this study, we analyzed the complexity of the peripheral blood T cell repertoire with a sensitive b-variable (BV) complementarity-determining region 3 (CDR3) spectratyping analysis and flow cytometry in three-stage IV CTCL/Sezary syndrome patients who achieved complete clinical remission after therapy. "
3.	Interferon-alpha (Interferon Alfa)FDA Link 03/01/2004 - "Thrombotic microangiopathy in a patient with Sezary syndrome treated with interferon-alpha." 12/01/1986 - "For patients with cutaneous T cell lymphoma (CTCL) (mycosis fungoides and the Sezary syndrome), a response rate of 45%, with 10% complete responses, was seen in patients treated with recombinant alpha-interferon at the National Cancer Institute (NCI). " 04/01/2011 - "The efficacy of vorinostat in combination with interferon alpha and extracorporeal photopheresis in late stage mycosis fungoides and Sezary syndrome." 01/01/1999 - "Bone marrow transplantation (BMT) from a matched unrelated donor was performed in a 22-year-old woman with a 10-year history of Sezary syndrome who had failed treatment with corticosteroids, methotrexate, photochemotherapy, photopheresis, hydroxyurea, interferon-alpha, and cladarabine. "
4.	bexarotene (LGD1069)FDA Link 09/01/2007 - "Our results show that bexarotene may inhibit malignant cell trafficking to the skin through an ability to suppress CCR4 expression among Sezary syndrome lymphocytes." 09/01/2007 - "To evaluate the effects of bexarotene on skin trafficking molecule expression and chemotaxis, peripheral blood mononuclear cells from Sezary syndrome patients and healthy controls were treated with bexarotene in vitro. " 09/01/2007 - "Bexarotene blunts malignant T-cell chemotaxis in Sezary syndrome: reduction of chemokine receptor 4-positive lymphocytes and decreased chemotaxis to thymus and activation-regulated chemokine." 05/01/2007 - "Apoptotic responses to all-trans retinoic acid of targretin-resistant, malignant, CD4+ peripheral blood T cells from patients with Sezary syndrome." 07/01/2013 - "Pentostatin plus cyclophosphamide and bexarotene is an effective and safe combination in patients with mycosis fungoides/Sezary syndrome."
5.	Pentostatin (Nipent)FDA LinkGeneric 08/01/1999 - "We present a case of a patient who developed all manifestations of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) acutely following treatment of cutaneous T-cell lymphoma (CTCL, Sezary syndrome) with deoxycoformycin (pentostatin). " 08/01/1999 - "Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) following treatment with deoxycoformycin in a patient with cutaneous T-cell lymphoma (Sezary syndrome): A case report." 07/01/2013 - "Pentostatin plus cyclophosphamide and bexarotene is an effective and safe combination in patients with mycosis fungoides/Sezary syndrome."
6.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 06/01/2013 - "We describe a 55-year-old woman suffering from Sezary syndrome, had undergone chemotherapy consisting of cycles of cyclophosphamide and prednisone. " 07/01/2013 - "Pentostatin plus cyclophosphamide and bexarotene is an effective and safe combination in patients with mycosis fungoides/Sezary syndrome."
7.	erlotinib (CP 358,774)FDA Link 01/01/2014 - "A prospective study was carried out to evaluate the efficacy of this drug in refractory pruritus secondary to Sezary syndrome, and other authors have studied the effectiveness of aprepitant against pruritus, secondary to biological therapy with erlotinib. "
8.	aprepitant (Emend)FDA Link 01/01/2014 - "A prospective study was carried out to evaluate the efficacy of this drug in refractory pruritus secondary to Sezary syndrome, and other authors have studied the effectiveness of aprepitant against pruritus, secondary to biological therapy with erlotinib. "
9.	DNA (Deoxyribonucleic Acid)IBA 07/01/2000 - "In a series of 24 patients with chronic T-lymphoid disorders [13 T-prolymphocytic leukaemia (T-PLL) and 11 Sezary syndrome] we have studied (i) chromosome 17p abnormalities and p53 allele deletion by fluorescence in situ hybridization; (ii) mutation in the exons of the p53 gene by direct DNA sequencing; and (iii) p53 protein expression by immunocytochemistry and, in some cases, also by flow cytometry with DO-1, a monoclonal antibody to the p53 protein. " 01/01/1986 - "Detection of HTLV-I (human T-cell lymphotropic virus, type I) proviral DNA in leukemic cells from a French patient with Sezary syndrome." 09/01/2008 - "Some of the DNA copy number aberrations in SPTL, such as loss of 10q, 17p, and chromosome 19, overlap with those characteristic of common forms of CTCL (mycosis fungoides (MF) and Sezary syndrome (SS)), whereas 5q and 13q gains characterize SPTL. " 11/01/2004 - "Flow cytometric DNA ploidy analysis of peripheral blood from patients with sezary syndrome: detection of aneuploid neoplastic T cells in the blood is associated with large cell transformation in tissue." 12/01/2002 - "We report a case of Sezary syndrome with two abnormal CD4+ T-cell populations detected in the peripheral blood by flow cytometry immunophenotyping and DNA cell content, suggesting a biclonal T-cell lymphoproliferative disorder. "
10.	T-Cell Antigen Receptors (T-Cell Receptor)IBA 11/01/2003 - "The aim of the study was to evaluate the usefulness of the T-cell receptor (TCR) gamma gene rearrangement analysis in the diagnosis of mycosis fungoides (MF) and Sezary syndrome (SS). " 07/01/1994 - "Detection of clonal T-cell receptor gamma gene rearrangements in early mycosis fungoides/Sezary syndrome by polymerase chain reaction and denaturing gradient gel electrophoresis (PCR/DGGE)." 06/15/1992 - "We investigated the correlation between the detection of clonal rearrangement of the T-cell antigen receptor gene (TCRR) in lymph node tissue with histopathologic lymph node classification in 33 patients with mycosis fungoides with and without the Sezary Syndrome. " 08/01/2006 - "We have examined in detail the features of the T-cell receptor beta (TCRB) gene rearrangements in 20 individuals with well-defined stages of cutaneous T-cell lymphoma (CTCL) comprising 10 cases with early-stage mycosis fungoides (MF) and 10 cases with late-stage MF or Sezary syndrome. " 07/01/1994 - "We used a gene amplification strategy to analyze T-cell receptor (TCR) gene rearrangements in 185 specimens, including mycosis fungoides/Sezary syndrome (MF/SS), other cutaneous neoplasms, inflammatory dermatoses, reactive lymphoid tissues, and normal skin. "

Therapies and Procedures

1.	Photopheresis 01/01/2002 - "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. " 04/01/2012 - "Extracorporeal photochemotherapy (ECP) is an effective treatment modality for patients with erythrodermic myocosis fungoides (MF) and Sezary syndrome (SS). " 09/01/1999 - "Furthermore, a 20%-25% complete response rate with photopheresis alone, or in combination with other biologic response modifiers, has been obtained at our institution among patients with Sezary syndrome. " 01/01/1997 - "In patients with Sezary syndrome undergoing extracorporeal photochemotherapy it is currently thought that photodamage of a congruent number of pathogenic circulating T cells is a necessary condition for a specific anticlonal response to be induced against these cells by the immune system. " 08/01/1996 - "Peripheral blood T-cell subpopulations of Sezary syndrome patients, including the malignant clone, were equally susceptible to apoptosis subsequent to either photopheresis or PUVA treatment. "
2.	Transplants (Transplant) 02/01/2009 - "Sezary syndrome in relapse after reduced intensity allogeneic transplant successfully treated with donor lymphocyte infusion." 02/01/2010 - "Based on these effects, ECP is mainly used for treatment of Sezary syndrome, graft-versus-host disease, organ graft rejection and autoimmune diseases. " 08/01/2003 - "Extracorporeal photochemotherapy (ECP) has been shown to be effective in variety of pathologic diseases such as Sezary syndrome, autoimmune diseases, organ graft rejection and graft versus host disease. "
3.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 01/01/1999 - "Remission of refractory Sezary syndrome after bone marrow transplantation from a matched unrelated donor." 01/01/1999 - "Bone marrow transplantation (BMT) from a matched unrelated donor was performed in a 22-year-old woman with a 10-year history of Sezary syndrome who had failed treatment with corticosteroids, methotrexate, photochemotherapy, photopheresis, hydroxyurea, interferon-alpha, and cladarabine. "
4.	Biological Therapy 01/01/2014 - "A prospective study was carried out to evaluate the efficacy of this drug in refractory pruritus secondary to Sezary syndrome, and other authors have studied the effectiveness of aprepitant against pruritus, secondary to biological therapy with erlotinib. "
5.	Photochemotherapy (Photodynamic Therapy) 03/01/2015 - "We performed an extensive search of published literature using keywords like "phototherapy", "photochemotherapy", "NBUVB", "PUVA", "UVA1", "mycosis fungoides", and "Sezary syndrome", and included systematic reviews, meta-analysis, national guidelines, randomized controlled trials (RCTs), prospective open label studies, and retrospective case series. " 01/01/2010 - "Photodynamic therapy with the silicon phthalocyanine pc 4 induces apoptosis in mycosis fungoides and sezary syndrome." 01/01/1999 - "Bone marrow transplantation (BMT) from a matched unrelated donor was performed in a 22-year-old woman with a 10-year history of Sezary syndrome who had failed treatment with corticosteroids, methotrexate, photochemotherapy, photopheresis, hydroxyurea, interferon-alpha, and cladarabine. "