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Sezary syndrome

MedGen UID:
19959
Concept ID:
C0036920
Neoplastic Process
Synonyms: SEZARY SYNDROME, SOMATIC; Sezary's lymphoma
SNOMED CT: Sezary syndrome (4950009); Sézary's syndrome (4950009); Sézary's disease (4950009); Sézary's disease (118611004); Sezary's disease (118611004); Sézary disease (4950009); Sézary disease (118611004); Sezary's disease (4950009)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0017844
Orphanet: ORPHA3162

Definition

Sézary syndrome is an aggressive form of a type of blood cancer called cutaneous T-cell lymphoma. Cutaneous T-cell lymphomas occur when certain white blood cells, called T cells, become cancerous; these cancers characteristically affect the skin, causing different types of skin lesions. In Sézary syndrome, the cancerous T cells, called Sézary cells, are present in the blood, skin, and lymph nodes. A characteristic of Sézary cells is an abnormally shaped nucleus, described as cerebriform.

People with Sézary syndrome develop a red, severely itchy rash (erythroderma) that covers large portions of their body. Sézary cells are found in the rash. However, the skin cells themselves are not cancerous; the skin problems result when Sézary cells move from the blood into the skin. People with Sézary syndrome also have enlarged lymph nodes (lymphadenopathy). Other common signs and symptoms of this condition include hair loss (alopecia), skin swelling (edema), thickened skin on the palms of the hands and soles of the feet (palmoplantar keratoderma), abnormalities of the fingernails and toenails, and lower eyelids that turn outward (ectropion). Some people with Sézary syndrome are less able to control their body temperature than people without the condition.

The cancerous T cells can spread to other organs in the body, including the lymph nodes, liver, spleen, and bone marrow. In addition, affected individuals have an increased risk of developing another lymphoma or other type of cancer.

Sézary syndrome most often occurs in adults over age 60 and usually progresses rapidly; historically, affected individuals survived an average of 2 to 4 years after development of the condition, although survival has improved with newer treatments.

Although Sézary syndrome is sometimes referred to as a variant of another cutaneous T-cell lymphoma called mycosis fungoides, these two cancers are generally considered separate conditions. [from MedlinePlus Genetics]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Sezary syndrome
Follow this link to review classifications for Sezary syndrome in Orphanet.

Professional guidelines

PubMed

Latzka J, Assaf C, Bagot M, Cozzio A, Dummer R, Guenova E, Gniadecki R, Hodak E, Jonak C, Klemke CD, Knobler R, Morrris S, Nicolay JP, Ortiz-Romero PL, Papadavid E, Pimpinelli N, Quaglino P, Ranki A, Scarisbrick J, Stadler R, Väkevä L, Vermeer MH, Wehkamp U, Whittaker S, Willemze R, Trautinger F
Eur J Cancer 2023 Dec;195:113343. Epub 2023 Sep 18 doi: 10.1016/j.ejca.2023.113343. PMID: 37890355
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Jan;98(1):193-209. Epub 2022 Oct 20 doi: 10.1002/ajh.26760. PMID: 36226409Free PMC Article
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2019 Sep;94(9):1027-1041. Epub 2019 Jul 31 doi: 10.1002/ajh.25577. PMID: 31313347

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Primary Cutaneous Lymphomas, 2024

Recent clinical studies

Etiology

Latzka J, Assaf C, Bagot M, Cozzio A, Dummer R, Guenova E, Gniadecki R, Hodak E, Jonak C, Klemke CD, Knobler R, Morrris S, Nicolay JP, Ortiz-Romero PL, Papadavid E, Pimpinelli N, Quaglino P, Ranki A, Scarisbrick J, Stadler R, Väkevä L, Vermeer MH, Wehkamp U, Whittaker S, Willemze R, Trautinger F
Eur J Cancer 2023 Dec;195:113343. Epub 2023 Sep 18 doi: 10.1016/j.ejca.2023.113343. PMID: 37890355
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Jan;98(1):193-209. Epub 2022 Oct 20 doi: 10.1002/ajh.26760. PMID: 36226409Free PMC Article
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2019 Sep;94(9):1027-1041. Epub 2019 Jul 31 doi: 10.1002/ajh.25577. PMID: 31313347
Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators
Lancet Oncol 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9 doi: 10.1016/S1470-2045(18)30379-6. PMID: 30100375
Pulitzer M
Clin Lab Med 2017 Sep;37(3):527-546. doi: 10.1016/j.cll.2017.06.006. PMID: 28802499Free PMC Article

Diagnosis

Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Jan;98(1):193-209. Epub 2022 Oct 20 doi: 10.1002/ajh.26760. PMID: 36226409Free PMC Article
Hague C, Farquharson N, Menasce L, Parry E, Cowan R
Br J Hosp Med (Lond) 2022 Apr 2;83(4):1-7. Epub 2022 Apr 14 doi: 10.12968/hmed.2021.0149. PMID: 35506718
Kamijo H, Miyagaki T
Curr Treat Options Oncol 2021 Jan 7;22(2):10. doi: 10.1007/s11864-020-00809-w. PMID: 33415447
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2019 Sep;94(9):1027-1041. Epub 2019 Jul 31 doi: 10.1002/ajh.25577. PMID: 31313347
Larocca C, Kupper T
Hematol Oncol Clin North Am 2019 Feb;33(1):103-120. doi: 10.1016/j.hoc.2018.09.001. PMID: 30497668Free PMC Article

Therapy

Latzka J, Assaf C, Bagot M, Cozzio A, Dummer R, Guenova E, Gniadecki R, Hodak E, Jonak C, Klemke CD, Knobler R, Morrris S, Nicolay JP, Ortiz-Romero PL, Papadavid E, Pimpinelli N, Quaglino P, Ranki A, Scarisbrick J, Stadler R, Väkevä L, Vermeer MH, Wehkamp U, Whittaker S, Willemze R, Trautinger F
Eur J Cancer 2023 Dec;195:113343. Epub 2023 Sep 18 doi: 10.1016/j.ejca.2023.113343. PMID: 37890355
Kamijo H, Miyagaki T
Curr Treat Options Oncol 2021 Jan 7;22(2):10. doi: 10.1007/s11864-020-00809-w. PMID: 33415447
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2019 Sep;94(9):1027-1041. Epub 2019 Jul 31 doi: 10.1002/ajh.25577. PMID: 31313347
Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators
Lancet Oncol 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9 doi: 10.1016/S1470-2045(18)30379-6. PMID: 30100375
Trautinger F, Eder J, Assaf C, Bagot M, Cozzio A, Dummer R, Gniadecki R, Klemke CD, Ortiz-Romero PL, Papadavid E, Pimpinelli N, Quaglino P, Ranki A, Scarisbrick J, Stadler R, Väkevä L, Vermeer MH, Whittaker S, Willemze R, Knobler R
Eur J Cancer 2017 May;77:57-74. Epub 2017 Mar 31 doi: 10.1016/j.ejca.2017.02.027. PMID: 28365528

Prognosis

Latzka J, Assaf C, Bagot M, Cozzio A, Dummer R, Guenova E, Gniadecki R, Hodak E, Jonak C, Klemke CD, Knobler R, Morrris S, Nicolay JP, Ortiz-Romero PL, Papadavid E, Pimpinelli N, Quaglino P, Ranki A, Scarisbrick J, Stadler R, Väkevä L, Vermeer MH, Wehkamp U, Whittaker S, Willemze R, Trautinger F
Eur J Cancer 2023 Dec;195:113343. Epub 2023 Sep 18 doi: 10.1016/j.ejca.2023.113343. PMID: 37890355
Hague C, Farquharson N, Menasce L, Parry E, Cowan R
Br J Hosp Med (Lond) 2022 Apr 2;83(4):1-7. Epub 2022 Apr 14 doi: 10.12968/hmed.2021.0149. PMID: 35506718
Kamijo H, Miyagaki T
Curr Treat Options Oncol 2021 Jan 7;22(2):10. doi: 10.1007/s11864-020-00809-w. PMID: 33415447
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2019 Sep;94(9):1027-1041. Epub 2019 Jul 31 doi: 10.1002/ajh.25577. PMID: 31313347
Larocca C, Kupper T
Hematol Oncol Clin North Am 2019 Feb;33(1):103-120. doi: 10.1016/j.hoc.2018.09.001. PMID: 30497668Free PMC Article

Clinical prediction guides

de Masson A, Beylot-Barry M, Ram-Wolff C, Mear JB, Dalle S, d'Incan M, Ingen-Housz-Oro S, Orvain C, Abraham J, Dereure O, Charbonnier A, Cornillon J, Longvert C, Barete S, Boulinguez S, Wierzbicka-Hainaut E, Aubin F, Rubio MT, Bernard M, Schmidt-Tanguy A, Houot R, Pham-Ledard A, Michonneau D, Brice P, Labussière-Wallet H, Bouaziz JD, Grange F, Moins-Teisserenc H, Jondeau K, Michel L, Mourah S, Battistella M, Daguindau E, Loschi M, Picard A, Franck N, Maillard N, Huynh A, Nguyen S, Marçais A, Chaby G, Ceballos P, Le Corre Y, Maury S, Bay JO, Adamski H, Bachy E, Forcade E, Socié G, Bagot M, Chevret S, Peffault de Latour R; CUTALLO Investigators; Groupe Français d'Etude des Lymphomes Cutanés; Société Française de Greffe de Moëlle et Thérapie Cellulaire
Lancet 2023 Jun 10;401(10392):1941-1950. Epub 2023 Apr 24 doi: 10.1016/S0140-6736(23)00329-X. PMID: 37105210
Phillips D, Matusiak M, Gutierrez BR, Bhate SS, Barlow GL, Jiang S, Demeter J, Smythe KS, Pierce RH, Fling SP, Ramchurren N, Cheever MA, Goltsev Y, West RB, Khodadoust MS, Kim YH, Schürch CM, Nolan GP
Nat Commun 2021 Nov 18;12(1):6726. doi: 10.1038/s41467-021-26974-6. PMID: 34795254Free PMC Article
Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators
Lancet Oncol 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9 doi: 10.1016/S1470-2045(18)30379-6. PMID: 30100375
Rubio Gonzalez B, Zain J, Rosen ST, Querfeld C
Curr Opin Oncol 2016 Jan;28(1):88-96. doi: 10.1097/CCO.0000000000000243. PMID: 26632770
Zinzani PL, Ferreri AJ, Cerroni L
Crit Rev Oncol Hematol 2008 Feb;65(2):172-82. Epub 2007 Oct 22 doi: 10.1016/j.critrevonc.2007.08.004. PMID: 17950613

Recent systematic reviews

Goyal A, O'Leary D, Foss F
Bone Marrow Transplant 2024 Jan;59(1):41-51. Epub 2023 Oct 18 doi: 10.1038/s41409-023-02122-0. PMID: 37853164
Jfri A, Smith JS, Larocca C
J Am Acad Dermatol 2023 May;88(5):1164-1166. Epub 2022 Dec 5 doi: 10.1016/j.jaad.2022.12.001. PMID: 36481378
Grimm SE, Witlox W, Wolff R, Chalker A, Hiligsmann M, Wijnen B, Ahmadu C, Ryder S, Armstrong N, Duffy S, Syndikus I, Kleijnen J, Joore MA
Pharmacoeconomics 2022 May;40(5):509-518. Epub 2021 Oct 19 doi: 10.1007/s40273-021-01098-3. PMID: 34664200Free PMC Article
Ottevanger R, van Beugen S, Evers AWM, Willemze R, Vermeer MH, Quint KD
J Eur Acad Dermatol Venereol 2021 Dec;35(12):2377-2387. Epub 2021 Aug 14 doi: 10.1111/jdv.17570. PMID: 34331819Free PMC Article
Mao X, Lillington DM, Czepulkowski B, Russell-Jones R, Young BD, Whittaker S
Genes Chromosomes Cancer 2003 Mar;36(3):250-60. doi: 10.1002/gcc.10152. PMID: 12557225

Supplemental Content

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Primary Cutaneous Lymphomas, 2024

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