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Sézary disease

April 12, 2024

Sézary disease, or Sézary syndrome,[1] is a type of cutaneous T-cell lymphoma that was first described by Albert Sézary.[2] The affected T cells, known as Sézary's cells or Lutzner cells, have pathological quantities of mucopolysaccharides. Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.[3][4]

Sézary disease
Other namesSézary's disease, Sézary('s) syndrome
The bright red rash of Sezary syndrome
Pronunciation
SpecialtyOncology, dermatology

Signs and symptoms edit

 
Sézary cell: pleomorphic abnormal T cell with the characteristic cerebriform nuclei (Peripheral blood - MGG stain)

Sézary disease and mycosis fungoides are cutaneous T-cell lymphomas having a primary manifestation in the skin.[5] The disease's origin is a peripheral CD4+ T-lymphocyte,[3] although rarer CD8+/CD4- cases have been observed.[3] Epidermotropism (lymphocytes residing in the epidermis)[6] by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. Although the condition can affect people of all ages, it is commonly diagnosed in adults over age 60.[7][3] The dominant signs and symptoms of the disease are:

  1. Generalized erythroderma – redness of the skin[3]
  2. Lymphadenopathy – swollen, enlarged lymph nodes[3]
  3. Atypical T cells – malignant lymphocytes known as "Sézary cells" seen in the peripheral blood with typical cerebriform nuclei (brain-shaped, convoluted nuclei)[8][3]
  4. Hepatosplenomegaly– enlarged liver and spleen[9]
  5. Palmoplantar keratoderma – thickening of the palms of the hands, and soles of the feet[10][11]

Diagnosis edit

 
Sézary syndrome in a 61-year-old man presenting in 1972 with unrelenting itchiness of six months’ duration. On the right is his peripheral blood film stained with Periodic Acid-Schiff (PAS) showing a neoplastic T cell (Sézary cell).

Those who have Sézary disease often present skin lesions that do not heal with normal medication.[12] A blood test generally reveals any change in the levels of lymphocytes in the blood, which is often associated with a cutaneous T-cell lymphoma.[12] Finally, a biopsy of a skin lesion can be performed to rule out any other causes.[12]

The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences:[13]

  1. More monotonous cellular infiltrates (large, clustered atypical pagetoid cells) in Sézary syndrome
  2. Sometimes absent epidermotropism
  3. Increased lymph node involvement with infiltrates of Sézary syndrome.

Treatment edit

Treatment typically includes some combination of photodynamic therapy, radiation therapy, chemotherapy, and biologic therapy.[14]

Treatments are often used in combination with phototherapy and chemotherapy, though pure chemotherapy is rarely used today.[3] No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.[15]

Radiation therapy edit

A number of types of radiation therapy may be used including total skin electron therapy.[16] While this therapy does not generally result in systemic toxic effects it can produce side effects involving the skin.[16] It is only available at a few institutions.[16]

Chemotherapy edit

Romidepsin, vorinostat and a few others are a second-line drug for cutaneous T-cell lymphoma.[17] Mogamulizumab has been approved in Japan[18] and the United States.[19]

Epidemiology edit

In the Western population, there are around 3 cases of Sézary syndrome per 1,000,000 people.[3] Sézary disease is more common in males with a ratio of 2:1,[3] and the mean age of diagnosis is between 55 and 60 years of age.[3][9]

See also edit

References edit

  1. ^ Reference, Genetics Home. "Sézary syndrome". Genetics Home Reference.
  2. ^ Sézary's cell at Who Named It?
  3. ^ a b c d e f g h i j k Cuneo, A; Castoldi, GL (2005). "Mycosis fungoidses/Sezary's syndrome". Atlas Genet Cytogenet Oncol Haematol. 9 (3): 242–243. Retrieved 2008-02-15.
  4. ^ Thangavelu M, Finn WG, Yelavarthi KK, Roenigk Jr HH, Samuelson E, Peterson L, Kuzel TM, Rosen ST (May 1997). "Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome". Blood. 89 (9): 3371–7. doi:10.1182/blood.V89.9.3371. PMID 9129044.
  5. ^ Cerroni, Lorenzo; Kevin Gatter; Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 39. ISBN 978-1-4051-1376-2.
  6. ^ Mutasim, Diya F. (2015). "What is Dermatitis with Epidermotropism?". Practical Skin Pathology. Springer International Publishing. pp. 37–39. doi:10.1007/978-3-319-14729-1_8. ISBN 978-3-319-14728-4.
  7. ^ "Sezary syndrome". genetic and rare diseases information center. Retrieved 17 April 2018.
  8. ^ Park, HS; McIntosh, L; Braschi-Amirfarzan, M; Shinagare, AB; Krajewski, KM (January 2017). "T-Cell Non-Hodgkin Lymphomas: Spectrum of Disease and the Role of Imaging in the Management of Common Subtypes". Korean Journal of Radiology. 18 (1): 71–83. doi:10.3348/kjr.2017.18.1.71. PMC 5240486. PMID 28096719.
  9. ^ a b Lorincz, A. I. "Sezary syndrome". Retrieved 2008-02-15.
  10. ^ Fragkos, Konstantinos C. (2017). "Plantar keratoderma of Sézary syndrome". Clinical Case Reports. 5 (10): 1726–1727. doi:10.1002/ccr3.1168. ISSN 2050-0904. PMC 5628207. PMID 29026585.
  11. ^ Martin, Stephanie J.; Duvic, Madeleine (2012-10-01). "Prevalence and treatment of palmoplantar keratoderma and tinea pedis in patients with Sézary syndrome". International Journal of Dermatology. 51 (10): 1195–1198. doi:10.1111/j.1365-4632.2011.05204.x. ISSN 1365-4632. PMID 22994666. S2CID 44779503.
  12. ^ a b c "Diagnosis". Retrieved 2008-02-15.
  13. ^ Beigi, Pooya Khan Mohammad (2017). "Diagnosis and Management". Clinician's Guide to Mycosis Fungoides. Vol. 35. Springer International Publishing. pp. 13–18. doi:10.1007/978-3-319-47907-1_4. ISBN 9783319479064. PMC 1806090. PMID 13618707. {{cite book}}: |journal= ignored (help)
  14. ^ "Mycosis Fungoides (Including Sézary Syndrome) Treatment". National Cancer Institute. 7 September 2017. Retrieved 1 December 2017.
  15. ^ Cerroni, Lorenzo; Kevin Gatter; Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 41. ISBN 978-1-4051-1376-2.
  16. ^ a b c "Mycosis Fungoides (Including Sézary Syndrome) Treatment". National Cancer Institute. 12 July 2017. Retrieved 1 December 2017.
  17. ^ Jawed, SI; Myskowski, PL; Horwitz, S; Moskowitz, A; Querfeld, C (February 2014). "Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions". Journal of the American Academy of Dermatology. 70 (2): 223.e1–17, quiz 240–2. doi:10.1016/j.jaad.2013.08.033. PMID 24438970.
  18. ^ Subramaniam, Joshuan M.; Whiteside, Glenn; McKeage, Kate; Croxtall, Jamie C. (2012-06-18). "Mogamulizumab: first global approval". Drugs. 72 (9): 1293–1298. doi:10.2165/11631090-000000000-00000. ISSN 1179-1950. PMID 22686619.
  19. ^ "FDA approves mogamulizumab-kpkc for mycosis fungoides or Sézary syndrome". U.S. Food and Drug Administration. 2018-08-08. Retrieved 2020-08-04.

External links edit

  • Sezary Syndrome lymphoma information

April 12, 2024
sézary, disease, sézary, syndrome, type, cutaneous, cell, lymphoma, that, first, described, albert, sézary, affected, cells, known, sézary, cells, lutzner, cells, have, pathological, quantities, mucopolysaccharides, sometimes, considered, late, stage, mycosis,. Sezary disease or Sezary syndrome 1 is a type of cutaneous T cell lymphoma that was first described by Albert Sezary 2 The affected T cells known as Sezary s cells or Lutzner cells have pathological quantities of mucopolysaccharides Sezary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy 3 4 Sezary diseaseOther namesSezary s disease Sezary s syndromeThe bright red rash of Sezary syndromePronunciation ˌ s eɪ ˌ z ɑː ˈ r iː SpecialtyOncology dermatology Contents 1 Signs and symptoms 2 Diagnosis 3 Treatment 3 1 Radiation therapy 3 2 Chemotherapy 4 Epidemiology 5 See also 6 References 7 External linksSigns and symptoms edit nbsp Sezary cell pleomorphic abnormal T cell with the characteristic cerebriform nuclei Peripheral blood MGG stain Sezary disease and mycosis fungoides are cutaneous T cell lymphomas having a primary manifestation in the skin 5 The disease s origin is a peripheral CD4 T lymphocyte 3 although rarer CD8 CD4 cases have been observed 3 Epidermotropism lymphocytes residing in the epidermis 6 by neoplastic CD4 lymphocytes with the formation of Pautrier s microabscesses is the hallmark sign of the disease Although the condition can affect people of all ages it is commonly diagnosed in adults over age 60 7 3 The dominant signs and symptoms of the disease are Generalized erythroderma redness of the skin 3 Lymphadenopathy swollen enlarged lymph nodes 3 Atypical T cells malignant lymphocytes known as Sezary cells seen in the peripheral blood with typical cerebriform nuclei brain shaped convoluted nuclei 8 3 Hepatosplenomegaly enlarged liver and spleen 9 Palmoplantar keratoderma thickening of the palms of the hands and soles of the feet 10 11 Diagnosis edit nbsp Sezary syndrome in a 61 year old man presenting in 1972 with unrelenting itchiness of six months duration On the right is his peripheral blood film stained with Periodic Acid Schiff PAS showing a neoplastic T cell Sezary cell Those who have Sezary disease often present skin lesions that do not heal with normal medication 12 A blood test generally reveals any change in the levels of lymphocytes in the blood which is often associated with a cutaneous T cell lymphoma 12 Finally a biopsy of a skin lesion can be performed to rule out any other causes 12 The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences 13 More monotonous cellular infiltrates large clustered atypical pagetoid cells in Sezary syndrome Sometimes absent epidermotropism Increased lymph node involvement with infiltrates of Sezary syndrome Treatment editTreatment typically includes some combination of photodynamic therapy radiation therapy chemotherapy and biologic therapy 14 Treatments are often used in combination with phototherapy and chemotherapy though pure chemotherapy is rarely used today 3 No single treatment type has revealed clear cut benefits in comparison to others treatment for all cases remains problematic 15 Radiation therapy edit A number of types of radiation therapy may be used including total skin electron therapy 16 While this therapy does not generally result in systemic toxic effects it can produce side effects involving the skin 16 It is only available at a few institutions 16 Chemotherapy edit Romidepsin vorinostat and a few others are a second line drug for cutaneous T cell lymphoma 17 Mogamulizumab has been approved in Japan 18 and the United States 19 Epidemiology editIn the Western population there are around 3 cases of Sezary syndrome per 1 000 000 people 3 Sezary disease is more common in males with a ratio of 2 1 3 and the mean age of diagnosis is between 55 and 60 years of age 3 9 See also editList of cutaneous conditionsReferences edit Reference Genetics Home Sezary syndrome Genetics Home Reference Sezary s cell at Who Named It a b c d e f g h i j k Cuneo A Castoldi GL 2005 Mycosis fungoidses Sezary s syndrome Atlas Genet Cytogenet Oncol Haematol 9 3 242 243 Retrieved 2008 02 15 Thangavelu M Finn WG Yelavarthi KK Roenigk Jr HH Samuelson E Peterson L Kuzel TM Rosen ST May 1997 Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides Sezary Syndrome Blood 89 9 3371 7 doi 10 1182 blood V89 9 3371 PMID 9129044 Cerroni Lorenzo Kevin Gatter Helmut Kerl 2005 An illustrated guide to Skin Lymphomas Malden Massachusetts Blackwell Publishing p 39 ISBN 978 1 4051 1376 2 Mutasim Diya F 2015 What is Dermatitis with Epidermotropism Practical Skin Pathology Springer International Publishing pp 37 39 doi 10 1007 978 3 319 14729 1 8 ISBN 978 3 319 14728 4 Sezary syndrome genetic and rare diseases information center Retrieved 17 April 2018 Park HS McIntosh L Braschi Amirfarzan M Shinagare AB Krajewski KM January 2017 T Cell Non Hodgkin Lymphomas Spectrum of Disease and the Role of Imaging in the Management of Common Subtypes Korean Journal of Radiology 18 1 71 83 doi 10 3348 kjr 2017 18 1 71 PMC 5240486 PMID 28096719 a b Lorincz A I Sezary syndrome Retrieved 2008 02 15 Fragkos Konstantinos C 2017 Plantar keratoderma of Sezary syndrome Clinical Case Reports 5 10 1726 1727 doi 10 1002 ccr3 1168 ISSN 2050 0904 PMC 5628207 PMID 29026585 Martin Stephanie J Duvic Madeleine 2012 10 01 Prevalence and treatment of palmoplantar keratoderma and tinea pedis in patients with Sezary syndrome International Journal of Dermatology 51 10 1195 1198 doi 10 1111 j 1365 4632 2011 05204 x ISSN 1365 4632 PMID 22994666 S2CID 44779503 a b c Diagnosis Retrieved 2008 02 15 Beigi Pooya Khan Mohammad 2017 Diagnosis and Management Clinician s Guide to Mycosis Fungoides Vol 35 Springer International Publishing pp 13 18 doi 10 1007 978 3 319 47907 1 4 ISBN 9783319479064 PMC 1806090 PMID 13618707 a href Template Cite book html title Template Cite book cite book a journal ignored help Mycosis Fungoides Including Sezary Syndrome Treatment National Cancer Institute 7 September 2017 Retrieved 1 December 2017 Cerroni Lorenzo Kevin Gatter Helmut Kerl 2005 An illustrated guide to Skin Lymphomas Malden Massachusetts Blackwell Publishing p 41 ISBN 978 1 4051 1376 2 a b c Mycosis Fungoides Including Sezary Syndrome Treatment National Cancer Institute 12 July 2017 Retrieved 1 December 2017 Jawed SI Myskowski PL Horwitz S Moskowitz A Querfeld C February 2014 Primary cutaneous T cell lymphoma mycosis fungoides and Sezary syndrome part II Prognosis management and future directions Journal of the American Academy of Dermatology 70 2 223 e1 17 quiz 240 2 doi 10 1016 j jaad 2013 08 033 PMID 24438970 Subramaniam Joshuan M Whiteside Glenn McKeage Kate Croxtall Jamie C 2012 06 18 Mogamulizumab first global approval Drugs 72 9 1293 1298 doi 10 2165 11631090 000000000 00000 ISSN 1179 1950 PMID 22686619 FDA approves mogamulizumab kpkc for mycosis fungoides or Sezary syndrome U S Food and Drug Administration 2018 08 08 Retrieved 2020 08 04 External links editSezary Syndrome lymphoma information Retrieved from https en wikipedia org w index php title Sezary disease amp oldid 1203853265, wikipedia, wiki, book, books, library,

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