sezary syndrome

Summary

Summary: 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).

Top Publications

  1. Bensussan A, Remtoula N, Sivori S, Bagot M, Moretta A, Marie Cardine A. Expression and function of the natural cytotoxicity receptor NKp46 on circulating malignant CD4+ T lymphocytes of Sézary syndrome patients. J Invest Dermatol. 2011;131:969-76 pubmed publisher
  2. Wysocka M, Benoit B, Newton S, Azzoni L, Montaner L, Rook A. Enhancement of the host immune responses in cutaneous T-cell lymphoma by CpG oligodeoxynucleotides and IL-15. Blood. 2004;104:4142-9 pubmed
    ..These findings have important implications for the potential enhancement of antitumor immunity among patients with advanced CTCL...
  3. Ballabio E, Mitchell T, van Kester M, Taylor S, Dunlop H, Chi J, et al. MicroRNA expression in Sezary syndrome: identification, function, and diagnostic potential. Blood. 2010;116:1105-13 pubmed publisher
    ..We also provide the first in vivo evidence for down-regulation of the miR-17-92 cluster in malignancy and demonstrate that ectopic miR-17-5p expression increases apoptosis and decreases cell proliferation in SzS cells...
  4. Samimi S, Benoit B, Evans K, Wherry E, Showe L, Wysocka M, et al. Increased programmed death-1 expression on CD4+ T cells in cutaneous T-cell lymphoma: implications for immune suppression. Arch Dermatol. 2010;146:1382-8 pubmed publisher
  5. Sibaud V, Beylot Barry M, Thiebaut R, Parrens M, Vergier B, Delaunay M, et al. Bone marrow histopathologic and molecular staging in epidermotropic T-cell lymphomas. Am J Clin Pathol. 2003;119:414-23 pubmed
    ..No further prognostic value was observed for the presence of a T-cell clone in bone marrow specimens. Our data do not support the need for bone marrow examination in patients with mycosis fungoides/Sézary syndrome. ..
  6. Gupta R, Ramble J, Tong C, Whittaker S, MacMahon E. Cytomegalovirus seroprevalence is not higher in patients with mycosis fungoides/Sezary syndrome. Blood. 2006;107:1241-2 pubmed
  7. van der Fits L, van Kester M, Qin Y, Out Luiting J, Smit F, Zoutman W, et al. MicroRNA-21 expression in CD4+ T cells is regulated by STAT3 and is pathologically involved in Sézary syndrome. J Invest Dermatol. 2011;131:762-8 pubmed publisher
    ..Silencing of miR-21 in Sézary cells results in increased apoptosis, suggesting a functional role for miR-21 in the leukomogenic process. Consequently, miR-21 might represent a therapeutic target for the treatment of SS...
  8. Kamstrup M, Gjerdrum L, Biskup E, Lauenborg B, Ralfkiaer E, Woetmann A, et al. Notch1 as a potential therapeutic target in cutaneous T-cell lymphoma. Blood. 2010;116:2504-12 pubmed publisher
    ..Our data show that Notch is present in cutaneous T-cell lymphoma and that its inhibition may provide a new way to treat cutaneous T-cell lymphoma...
  9. Olsen E, Rook A, Zic J, Kim Y, Porcu P, Querfeld C, et al. Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol. 2011;64:352-404 pubmed publisher
    ..Finally, guidelines for the treatment of SS and suggestions for adjuvant treatment are noted...

More Information

Publications64

  1. Scala E, Abeni D, Pomponi D, Narducci M, Lombardo G, Mari A, et al. The role of 9-O-acetylated ganglioside D3 (CD60) and {alpha}4{beta}1 (CD49d) expression in predicting the survival of patients with Sezary syndrome. Haematologica. 2010;95:1905-12 pubmed publisher
  2. Introcaso C, Hess S, Kamoun M, Ubriani R, Gelfand J, Rook A. Association of change in clinical status and change in the percentage of the CD4+CD26- lymphocyte population in patients with Sézary syndrome. J Am Acad Dermatol. 2005;53:428-34 pubmed
    ..It has been our clinical experience that changes in patients' CD4+CD26- T-cell populations of peripheral blood lymphocytes herald changes in their clinical status...
  3. Querfeld C, Rosen S, Guitart J, Rademaker A, Pezen D, Dolan M, et al. Multicenter phase II trial of temozolomide in mycosis fungoides/sezary syndrome: correlation with O?-methylguanine-DNA methyltransferase and mismatch repair proteins. Clin Cancer Res. 2011;17:5748-54 pubmed publisher
    ..Malignant CD4(+) T cells of patients with mycosis fungoides/Sézary syndrome (MF/SS) have been shown to have low levels of MGMT and may be particularly sensitive to this methylator...
  4. Contassot E, Kerl K, Roques S, Shane R, Gaide O, Dupuis M, et al. Resistance to FasL and tumor necrosis factor-related apoptosis-inducing ligand-mediated apoptosis in Sezary syndrome T-cells associated with impaired death receptor and FLICE-inhibitory protein expression. Blood. 2008;111:4780-7 pubmed publisher
    ..Our data on primary SzS lymphocytes reveal frequent resistance to apoptosis induced by FasL and TRAIL, which may contribute to their accumulation in patients with SzS and be relevant at a therapeutic level...
  5. Krejsgaard T, Gjerdrum L, Ralfkiaer E, Lauenborg B, Eriksen K, Mathiesen A, et al. Malignant Tregs express low molecular splice forms of FOXP3 in Sézary syndrome. Leukemia. 2008;22:2230-9 pubmed publisher
    ..Furthermore, we provide evidence that FOXP3 splice forms are functionally different from wt FOXP3 and not involved in the execution of the suppressive function. Thus, this is the first description of FOXP3 splice forms in human disease...
  6. Vermeer M, van Doorn R, Dijkman R, Mao X, Whittaker S, van Voorst Vader P, et al. Novel and highly recurrent chromosomal alterations in Sézary syndrome. Cancer Res. 2008;68:2689-98 pubmed publisher
    ..Prominent among deregulated genes are those encoding cMYC, cMYC-regulating proteins, mediators of MYC-induced apoptosis, and IL-2 signaling pathway components...
  7. Sokolowska Wojdylo M, Wenzel J, Gaffal E, Steitz J, Roszkiewicz J, Bieber T, et al. Absence of CD26 expression on skin-homing CLA+ CD4+ T lymphocytes in peripheral blood is a highly sensitive marker for early diagnosis and therapeutic monitoring of patients with Sézary syndrome. Clin Exp Dermatol. 2005;30:702-6 pubmed
    ..We conclude that the absence of CD26 expression on skin-homing CLA+ CD4+ T-helper cells is a very sensitive and highly specific parameter for early diagnosis and therapeutic monitoring of patients with SS...
  8. Jones D, Dang N, Duvic M, Washington L, Huh Y. Absence of CD26 expression is a useful marker for diagnosis of T-cell lymphoma in peripheral blood. Am J Clin Pathol. 2001;115:885-92 pubmed
  9. Ringrose A, Zhou Y, Pang E, Zhou L, Lin A, Sheng G, et al. Evidence for an oncogenic role of AHI-1 in Sezary syndrome, a leukemic variant of human cutaneous T-cell lymphomas. Leukemia. 2006;20:1593-601 pubmed
    ..transformed properties, knockdown of AHI-1 expression in Hut78 cells, a cell line derived from a patient with Sezary syndrome (SS), was performed using retroviral-mediated RNA interference...
  10. Hwang S, Janik J, Jaffe E, Wilson W. Mycosis fungoides and Sézary syndrome. Lancet. 2008;371:945-57 pubmed publisher
  11. Krejsgaard T, Odum N, Geisler C, Wasik M, Woetmann A. Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome. Leukemia. 2012;26:424-32 pubmed publisher
    ..Here, we review the literature to clarify the role of Tregs in MF and SS and discuss the potential mechanisms driving the immunodeficiency...
  12. Chung J, Shiue L, Duvic M, Pandya A, Cruz P, Ariizumi K. Sézary syndrome cells overexpress syndecan-4 bearing distinct heparan sulfate moieties that suppress T-cell activation by binding DC-HIL and trapping TGF-beta on the cell surface. Blood. 2011;117:3382-90 pubmed publisher
    ..Finally, we showed toxin-conjugated DC-HIL to abrogate the ability of SS cells to proliferate in vitro. These findings indicate that SD-4 bearing distinct HS moieties plays a pathogenic role in SS and may be targeted for treatment...
  13. Jones C, Wain E, Chu C, Tosi I, Foster R, McKenzie R, et al. Downregulation of Fas gene expression in Sézary syndrome is associated with promoter hypermethylation. J Invest Dermatol. 2010;130:1116-25 pubmed publisher
    ..These data demonstrate that dysregulation of Fas expression is a common feature of SS, and provide a rationale for targeted therapies to restore the extrinsic Fas-dependent apoptotic pathway in this malignancy...
  14. Mao X, Orchard G, Lillington D, Russell Jones R, Young B, Whittaker S. Amplification and overexpression of JUNB is associated with primary cutaneous T-cell lymphomas. Blood. 2003;101:1513-9 pubmed
    ..gains of RAF1 (3p25), CTSB (8p22), PAK1 (11q13), and JUNB (19p13) in 5 of 7 cases of mycosis fungoides (MF)/Sezary syndrome (SS) (71%), gains of FGFR1 (8p11), PTPN (20q13), and BCR (22q11) in 4 cases (57%), and gains of MYCL1 (1p34), ..
  15. Tsai E, Taur A, Espinosa L, Quon A, Johnson D, Dick S, et al. Staging accuracy in mycosis fungoides and sezary syndrome using integrated positron emission tomography and computed tomography. Arch Dermatol. 2006;142:577-84 pubmed
    ..To evaluate the usefulness of integrated positron emission tomography and computed tomography (PET/CT) in staging mycosis fungoides (MF) and Sézary syndrome and to correlate PET/CT data with histopathologic diagnosis of lymph nodes (LNs)...
  16. Duvic M, Donato M, Dabaja B, Richmond H, Singh L, Wei W, et al. Total skin electron beam and non-myeloablative allogeneic hematopoietic stem-cell transplantation in advanced mycosis fungoides and Sezary syndrome. J Clin Oncol. 2010;28:2365-72 pubmed publisher
    ..We studied the safety and efficacy of total skin electron beam with allogeneic hematopoietic stem-cell transplantation (HSCT) in patients with cutaneous T-cell lymphoma (CTCL)...
  17. Ortonne N, Huet D, Gaudez C, Marie Cardine A, Schiavon V, Bagot M, et al. Significance of circulating T-cell clones in Sezary syndrome. Blood. 2006;107:4030-8 pubmed
    ..Identification of the malignant cells in Sézary syndrome cannot be achieved by T-cell clonality studies or by TCR Vbeta monoclonal antibody (mAb) analysis alone; it also relies on CD158k phenotyping...
  18. Scarisbrick J, Woolford A, Calonje E, Photiou A, Ferreira S, Orchard G, et al. Frequent abnormalities of the p15 and p16 genes in mycosis fungoides and sezary syndrome. J Invest Dermatol. 2002;118:493-9 pubmed
    ..loss on 9p, 10q, and 17p including losses on 9p21 in 16% of patients with mycosis fungoides and 46% with Sezary syndrome. The P15 and P16 genes are intricately linked on 9p21 and can be inactivated in melanoma and non-Hodgkin's ..
  19. Ciree A, Michel L, Camilleri Broet S, Jean Louis F, Oster M, Flageul B, et al. Expression and activity of IL-17 in cutaneous T-cell lymphomas (mycosis fungoides and Sezary syndrome). Int J Cancer. 2004;112:113-20 pubmed
    ..in human tumors, we look for the natural expression and activity of IL-17 in mycosis fungoides (MF) and Sezary syndrome (SS)...
  20. Kari L, Loboda A, Nebozhyn M, Rook A, Vonderheid E, Nichols C, et al. Classification and prediction of survival in patients with the leukemic phase of cutaneous T cell lymphoma. J Exp Med. 2003;197:1477-88 pubmed
    ..peripheral blood mononuclear cells from patients with leukemic forms of cutaneous T cell lymphoma, primarily Sezary syndrome (SS)...
  21. Mitchell T, Whittaker S, John S. Dysregulated expression of COOH-terminally truncated Stat5 and loss of IL2-inducible Stat5-dependent gene expression in Sezary Syndrome. Cancer Res. 2003;63:9048-54 pubmed
    b>Sezary Syndrome (SzS) is a leukemic variant of cutaneous T-cell lymphoma characterized by the accumulation of clonal neoplastic CD4+ T cells...
  22. Politou M, Tsaftarides P, Vassiliades J, Siakantaris M, Michail S, Nakopoulou L, et al. Thrombotic microangiopathy in a patient with Sezary syndrome treated with interferon-alpha. Nephrol Dial Transplant. 2004;19:733-5 pubmed
  23. Wong H, Mishra A, Hake T, Porcu P. Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome). Br J Haematol. 2011;155:150-66 pubmed publisher
    ..The most common forms of CTCL are Mycosis Fungoides (MF) and Sezary Syndrome (SS)...
  24. Trautinger F, Knobler R, Willemze R, Peris K, Stadler R, Laroche L, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. Eur J Cancer. 2006;42:1014-30 pubmed
    ..Patients with advanced disease should be encouraged to participate in clinical trials and maintenance of quality of life should be paramount...
  25. Eriksen K, Kaltoft K, Mikkelsen G, Nielsen M, Zhang Q, Geisler C, et al. Constitutive STAT3-activation in Sezary syndrome: tyrphostin AG490 inhibits STAT3-activation, interleukin-2 receptor expression and growth of leukemic Sezary cells. Leukemia. 2001;15:787-93 pubmed
    ..of IL-2Ralpha expression and STATs in a tumor cell line obtained from peripheral blood from a patient with Sezary syndrome (SS), a leukemic variant of CTCL...
  26. Cerroni L, Fink Puches R, Bäck B, Kerl H. Follicular mucinosis: a critical reappraisal of clinicopathologic features and association with mycosis fungoides and Sézary syndrome. Arch Dermatol. 2002;138:182-9 pubmed
  27. Sokolowska Wojdylo M, Wenzel J, Gaffal E, Lenz J, Speuser P, Erdmann S, et al. Circulating clonal CLA(+) and CD4(+) T cells in Sezary syndrome express the skin-homing chemokine receptors CCR4 and CCR10 as well as the lymph node-homing chemokine receptor CCR7. Br J Dermatol. 2005;152:258-64 pubmed
    ..It has recently been reported that the chemokine CCL27 expressed by keratinocytes attracts lymphocytes bearing the chemokine receptor CCR10...
  28. Parker S, Bethaney J. Cutaneous T cell lymphoma-mycosis fungoides and Sezary syndrome: an update. G Ital Dermatol Venereol. 2009;144:467-85 pubmed
    ..MF and Sezary syndrome (SS) are now separately classified as indolent and aggressive CTCLs, respectively...
  29. Benner M, Jansen P, Vermeer M, Willemze R. Prognostic factors in transformed mycosis fungoides: a retrospective analysis of 100 cases. Blood. 2012;119:1643-9 pubmed publisher
    ..001). This prognostic index may be helpful in predicting prognosis and selecting the most appropriate treatment in patients with transformed MF...
  30. Narducci M, Scala E, Bresin A, Caprini E, Picchio M, Remotti D, et al. Skin homing of Sézary cells involves SDF-1-CXCR4 signaling and down-regulation of CD26/dipeptidylpeptidase IV. Blood. 2006;107:1108-15 pubmed
    ..Our findings suggest that the SDF-1-CXCR4 axis could play an important role in skin homing of SS through the regulatory activity of CD26...
  31. Nobbe S, Dziunycz P, Mühleisen B, Bilsborough J, Dillon S, French L, et al. IL-31 expression by inflammatory cells is preferentially elevated in atopic dermatitis. Acta Derm Venereol. 2012;92:24-8 pubmed publisher
    ..Our results confirm, at the protein level, the relationship between IL-31 expression and atopic dermatitis. Our results do not support a general relationship between expression of IL-31/IL-31R and pruritic or Th2-mediated diseases...
  32. Pai R, Mullins F, Kim Y, Kong C. Cytologic evaluation of lymphadenopathy associated with mycosis fungoides and Sezary syndrome: role of immunophenotypic and molecular ancillary studies. Cancer. 2008;114:323-32 pubmed publisher
    The most common presenting site of extracutaneous disease in mycosis fungoides and Sezary syndrome is the peripheral lymph node...
  33. Poszepczynska Guigné E, Schiavon V, D Incan M, Echchakir H, Musette P, Ortonne N, et al. CD158k/KIR3DL2 is a new phenotypic marker of Sezary cells: relevance for the diagnosis and follow-up of Sezary syndrome. J Invest Dermatol. 2004;122:820-3 pubmed
    ..of atypical circulating cells (Sezary cells) determined by cytomorphology in a large group of patients with Sezary syndrome. Moreover, we show that circulating CD4+CD158k+ lymphocytes correspond to the malignant clonal cell ..
  34. Raphael B, Shin D, Suchin K, Morrissey K, Vittorio C, Kim E, et al. High clinical response rate of Sezary syndrome to immunomodulatory therapies: prognostic markers of response. Arch Dermatol. 2011;147:1410-5 pubmed publisher
    ..To quantify response rates of Sézary syndrome (SS) to multimodality immunomodulatory therapy and to identify the important prognostic parameters that affect overall response to treatment...
  35. Jacobsen E, Kim H, Ho V, Cutler C, Koreth J, Fisher D, et al. A large single-center experience with allogeneic stem-cell transplantation for peripheral T-cell non-Hodgkin lymphoma and advanced mycosis fungoides/Sezary syndrome. Ann Oncol. 2011;22:1608-13 pubmed publisher
    ..The prognosis for patients with most forms of T-cell lymphoma is poor. Allogeneic hematopoietic stem-cell transplantation (HSCT) may improve the outcome...
  36. Lundin J, Hagberg H, Repp R, Cavallin Stahl E, Freden S, Juliusson G, et al. Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood. 2003;101:4267-72 pubmed
    ..Alemtuzumab shows promising clinical activity and an acceptable safety profile in patients with advanced MF/SS, particularly in patients with erythroderma and severe itching and those who were not heavily pretreated...
  37. Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment . Blood. 2007;110:1713-22 pubmed
    ..The evidence supporting the revisions is discussed as well as recommendations for evaluation and staging procedures based on these revisions...
  38. Wain E, Mitchell T, Russell Jones R, Whittaker S. Fine mapping of chromosome 10q deletions in mycosis fungoides and sezary syndrome: identification of two discrete regions of deletion at 10q23.33-24.1 and 10q24.33-25.1. Genes Chromosomes Cancer. 2005;42:184-92 pubmed
    Previous cytogenetic studies in mycosis fungoides (MF) and Sezary syndrome (SS) have identified a large and poorly defined area of chromosomal deletion on chromosome 10q...
  39. Wu J, Nihal M, Siddiqui J, Vonderheid E, Wood G. Low FAS/CD95 expression by CTCL correlates with reduced sensitivity to apoptosis that can be restored by FAS upregulation. J Invest Dermatol. 2009;129:1165-73 pubmed publisher
    FAS expression was generally low in 30 of 31 cutaneous T-cell lymphoma (CTCL) cases (mycosis fungoides/Sezary syndrome, SS) as well as in 5 of 6 large plaque parapsoriasis cases (a CTCL precursor)...
  40. van Doorn R, van Kester M, Dijkman R, Vermeer M, Mulder A, Szuhai K, et al. Oncogenomic analysis of mycosis fungoides reveals major differences with Sezary syndrome. Blood. 2009;113:127-36 pubmed publisher
    ..Furthermore, our analysis uncovered genomic differences between MF and Sz, which suggest that the molecular pathogenesis and therefore therapeutic requirements of these cutaneous T-cell lymphomas may be distinct...
  41. Su M, Dorocicz I, Dragowska W, Ho V, Li G, Voss N, et al. Aberrant expression of T-plastin in Sezary cells. Cancer Res. 2003;63:7122-7 pubmed
    Mycosis fungoides (MF) and its leukemic variant, Sezary syndrome (SS), are the most common cutaneous T-cell lymphomas, with a combined incidence of 0.36 of 100,000 person-years...
  42. Molina A, Zain J, Arber D, Angelopolou M, O Donnell M, Murata Collins J, et al. Durable clinical, cytogenetic, and molecular remissions after allogeneic hematopoietic cell transplantation for refractory Sezary syndrome and mycosis fungoides. J Clin Oncol. 2005;23:6163-71 pubmed
    b>Sezary syndrome (SS) and tumor-stage mycosis fungoides (MF) are generally incurable with currently available treatments...
  43. Bernengo M, Novelli M, Quaglino P, Lisa F, De Matteis A, Savoia P, et al. The relevance of the CD4+ CD26- subset in the identification of circulating Sézary cells. Br J Dermatol. 2001;144:125-35 pubmed
    ..In previous reports we showed that the lack of CD26 (dipeptidyl-aminopeptidase IV) is a characteristic feature of circulating Sézary cells (SC)...
  44. Laharanne E, Oumouhou N, Bonnet F, Carlotti M, Gentil C, Chevret E, et al. Genome-wide analysis of cutaneous T-cell lymphomas identifies three clinically relevant classes. J Invest Dermatol. 2010;130:1707-18 pubmed publisher
    ..Finally, different samples in the same patient clustered together, showing reproducibility of such a classifier...
  45. Caprini E, Cristofoletti C, Arcelli D, Fadda P, Citterich M, Sampogna F, et al. Identification of key regions and genes important in the pathogenesis of sezary syndrome by combining genomic and expression microarrays. Cancer Res. 2009;69:8438-46 pubmed publisher
    ..Altogether, our study confirms and maps more precisely the regions of gain and loss and, combined to transcriptional profiles, suggests a novel set of genes of potential interest in SS...
  46. van Doorn R, Dijkman R, Vermeer M, Out Luiting J, van der Raaij Helmer E, Willemze R, et al. Aberrant expression of the tyrosine kinase receptor EphA4 and the transcription factor twist in Sézary syndrome identified by gene expression analysis. Cancer Res. 2004;64:5578-86 pubmed
    ..Detection of EphA4 and Twist may be used in the molecular diagnosis of Sz and related cutaneous T-cell lymphomas. Furthermore, the membrane-bound EphA4 receptor may serve as a target for directed therapeutic intervention...
  47. Kim Y, Liu H, Mraz Gernhard S, Varghese A, Hoppe R. Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome: clinical prognostic factors and risk for disease progression. Arch Dermatol. 2003;139:857-66 pubmed
    ..To study and update the clinical characteristics and long-term outcome of our patients with mycosis fungoides (MF) and Sézary syndrome (SS), and to identify important clinical factors predictive of survival and disease progression...
  48. Prince H, Whittaker S, Hoppe R. How I treat mycosis fungoides and Sézary syndrome. Blood. 2009;114:4337-53 pubmed publisher
    ..It is appropriate to consider patients for novel agents within clinical trials if they have failed front-line therapy and before chemotherapy is used...
  49. Kim Y, Willemze R, Pimpinelli N, Whittaker S, Olsen E, Ranki A, et al. TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of R. Blood. 2007;110:479-84 pubmed
    ..Due to the clinical and pathologic heterogeneity of the cutaneous lymphomas, the currently proposed TNM system is meant to be primarily an anatomic documentation of disease extent and not to be used as a prognostic guide...
  50. Miyagaki T, Sugaya M. Erythrodermic cutaneous T-cell lymphoma: how to differentiate this rare disease from atopic dermatitis. J Dermatol Sci. 2011;64:1-6 pubmed publisher
    ..Older patients, high serum LDH levels, and high number of circulating atypical lymphocytes are associated with poor prognosis...
  51. Showe L, Fox F, Williams D, Au K, Niu Z, Rook A. Depressed IL-12-mediated signal transduction in T cells from patients with Sézary syndrome is associated with the absence of IL-12 receptor beta 2 mRNA and highly reduced levels of STAT4. J Immunol. 1999;163:4073-9 pubmed
    ..This is the first observation linking STAT4 gene expression with a human disease and suggests that dysregulation of STAT4 expression may be significant to the development and/or progression of SS...
  52. Campbell J, Clark R, Watanabe R, Kupper T. Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors. Blood. 2010;116:767-71 pubmed publisher
    ..Our results suggest that SS is a malignancy of central memory T cells and MF is a malignancy of skin resident effector memory T cells...
  53. Whittaker S, Foss F. Efficacy and tolerability of currently available therapies for the mycosis fungoides and Sezary syndrome variants of cutaneous T-cell lymphoma. Cancer Treat Rev. 2007;33:146-60 pubmed
    ..Cutaneous T-cell lymphoma variants include mycosis fungoides and Sezary syndrome, which are generally associated, respectively, with indolent and aggressive clinical courses and are the ..
  54. Kashani Sabet M, McMillan A, Zackheim H. A modified staging classification for cutaneous T-cell lymphoma. J Am Acad Dermatol. 2001;45:700-6 pubmed
    ..Despite refinements in the diagnosis of cutaneous T-cell lymphoma (CTCL), since 1979 there have been no changes to the staging of CTCL used to classify mycosis fungoides and Sézary syndrome...
  55. LEE C, Ungewickell A, Bhaduri A, Qu K, Webster D, Armstrong R, et al. Transcriptome sequencing in Sezary syndrome identifies Sezary cell and mycosis fungoides-associated lncRNAs and novel transcripts. Blood. 2012;120:3288-97 pubmed publisher
    ..Our findings characterize the SS transcriptome and support recent reports that implicate lncRNA dysregulation in human malignancies...