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Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers.
J Am Acad Dermatol 2014; 70(2):205.e1-16; quiz 221-2JA

Abstract

Mycosis fungoides (MF) and Sézary syndrome (SS) comprise approximately 53% of cutaneous lymphomas. Both MF and SS may clinically and histologically mimic benign skin conditions, posing a diagnostic challenge to the dermatologist. Precise clinicopathologic correlation is necessary to support a diagnosis, especially in the early stages of disease. In addition to the identification of histopathologic criteria, ancillary studies, including the identification of CD4(+) T cells with aberrant immunophenotypes and T-cell receptor gene rearrangements within skin lesions and peripheral blood are used to support the diagnosis. Recent studies evaluating the pathogenesis of MF have found that the skin microenvironment, including immune cells, such as dendritic cells and reactive cytotoxic and regulatory T cells, plays a crucial supporting role in MF. The skin-homing ability of malignant T cells is the result of chemokines, cytokines, adhesion molecules, and defective apoptosis, and is believed to play a role in disease pathogenesis and progression. In addition, recent studies have also suggested that MF and SS arise from distinct memory T cell subsets and advanced/erythrodermic MF and SS may be distinguished by identification of certain molecules, including Programmed-Death-1.

Authors+Show Affiliations

Dermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York.Dermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York.Lymphoma Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York.Lymphoma Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York.Dermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York. Electronic address: querfelc@mskcc.org.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

24438969

Citation

Jawed, Sarah I., et al. "Primary Cutaneous T-cell Lymphoma (mycosis Fungoides and Sézary Syndrome): Part I. Diagnosis: Clinical and Histopathologic Features and New Molecular and Biologic Markers." Journal of the American Academy of Dermatology, vol. 70, no. 2, 2014, pp. 205.e1-16; quiz 221-2.
Jawed SI, Myskowski PL, Horwitz S, et al. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. J Am Acad Dermatol. 2014;70(2):205.e1-16; quiz 221-2.
Jawed, S. I., Myskowski, P. L., Horwitz, S., Moskowitz, A., & Querfeld, C. (2014). Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. Journal of the American Academy of Dermatology, 70(2), pp. 205.e1-16; quiz 221-2. doi:10.1016/j.jaad.2013.07.049.
Jawed SI, et al. Primary Cutaneous T-cell Lymphoma (mycosis Fungoides and Sézary Syndrome): Part I. Diagnosis: Clinical and Histopathologic Features and New Molecular and Biologic Markers. J Am Acad Dermatol. 2014;70(2):205.e1-16; quiz 221-2. PubMed PMID: 24438969.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. AU - Jawed,Sarah I, AU - Myskowski,Patricia L, AU - Horwitz,Steven, AU - Moskowitz,Alison, AU - Querfeld,Christiane, PY - 2013/06/13/received PY - 2013/06/25/revised PY - 2013/07/01/accepted PY - 2014/1/21/entrez PY - 2014/1/21/pubmed PY - 2014/3/19/medline KW - APC KW - CLA KW - CTCL KW - E-CTCL KW - EMF KW - GMF KW - HTLV-1 KW - IL KW - ISCL KW - International Society for Cutaneous Lymphoma KW - LCT KW - MF KW - PD-1 KW - Programmed-Death-1 KW - SS KW - Sézary syndrome KW - T-cell receptor KW - TCR KW - Treg KW - antigen-presenting cell KW - biologic and molecular markers KW - clinical and diagnostic challenges KW - cutaneous T-cell lymphoma KW - cutaneous lymphocyte antigen KW - erythrodermic cutaneous T-cell lymphoma KW - erythrodermic mycosis fungoides KW - genetic aberrations KW - granulomatous mycosis fungoides KW - histopathology KW - human T-lymphotropic virus type 1 KW - interleukin KW - large cell transformation KW - mycosis fungoides KW - mycosis fungoides subtypes KW - pathogenesis KW - prognostic value KW - regulatory T cells KW - skin homing features KW - transformed mycosis fungoides KW - tumor microenvironment SP - 205.e1-16; quiz 221-2 JF - Journal of the American Academy of Dermatology JO - J. Am. Acad. Dermatol. VL - 70 IS - 2 N2 - Mycosis fungoides (MF) and Sézary syndrome (SS) comprise approximately 53% of cutaneous lymphomas. Both MF and SS may clinically and histologically mimic benign skin conditions, posing a diagnostic challenge to the dermatologist. Precise clinicopathologic correlation is necessary to support a diagnosis, especially in the early stages of disease. In addition to the identification of histopathologic criteria, ancillary studies, including the identification of CD4(+) T cells with aberrant immunophenotypes and T-cell receptor gene rearrangements within skin lesions and peripheral blood are used to support the diagnosis. Recent studies evaluating the pathogenesis of MF have found that the skin microenvironment, including immune cells, such as dendritic cells and reactive cytotoxic and regulatory T cells, plays a crucial supporting role in MF. The skin-homing ability of malignant T cells is the result of chemokines, cytokines, adhesion molecules, and defective apoptosis, and is believed to play a role in disease pathogenesis and progression. In addition, recent studies have also suggested that MF and SS arise from distinct memory T cell subsets and advanced/erythrodermic MF and SS may be distinguished by identification of certain molecules, including Programmed-Death-1. SN - 1097-6787 UR - https://www.unboundmedicine.com/medline/citation/24438969/Primary_cutaneous_T_cell_lymphoma__mycosis_fungoides_and_Sézary_syndrome_:_part_I__Diagnosis:_clinical_and_histopathologic_features_and_new_molecular_and_biologic_markers_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0190-9622(13)00914-6 DB - PRIME DP - Unbound Medicine ER -