Sézary disease

Sézary disease
Synonyms Sézary's disease, Sézary('s) syndrome
The bright red rash of Sezary syndrome
Pronunciation
  • /ˌseɪˌzɑːˈriː/
Classification and external resources
Specialty oncology, dermatology
ICD-10 C84.1
ICD-9-CM 202.2
ICD-O M9701/3
DiseasesDB 8595
eMedicine med/1541 derm/566 med/3486
MeSH D012751

Sézary disease is a type of cutaneous lymphoma that was first described by Albert Sézary.[1] The affected cells are T-cells (so it is a T-cell lymphoma) that have pathological quantities of mucopolysaccharides. Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.[2][3] There are currently no known causes of Sézary disease.[4]

Signs and symptoms

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

Sézary syndrome and mycosis fungoides are T-cell lymphomas whose primary manifestation is in the skin.[5] The disease's origin is a peripheral CD4+ T-lymphocyte,[2] although rarer CD8+/CD4- cases have been observed.[2] Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease.[2] The dominant symptoms of the disease are:

  1. Generalized erythroderma[2]
  2. Lymphadenopathy[2]
  3. Atypical T-cells ("Sézary cells") in the peripheral blood[2]
  4. Hepatosplenomegaly[6]

Diagnosis

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 with skin lesions that do not heal with normal medication.[7] 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.[7] Finally, a biopsy of a skin lesion can be performed to rule out any other causes.[7]

The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences[8]:

  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

Romidepsin, Vorinostat and a few others are a second-line drug for cutaneous T-cell lymphoma.[9] Mogamulizumab has been approved in Japan and waiting FDA approval in the United States. There are dozens of clinical trials, with a few in Phase III. Treatments are often used in combination with phototherapy and chemotherapy, though pure chemotherapy is rarely used today.[2] No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.[10] Stanford University has been pioneering low-dose radiation (1/3 of the standard[11]), followed by stem-cell transplantation without chemo, as a potential cure with promising results.[12]

Some centers view the condition with an holistic approach,[13] testing for infections,[14] food sensitives and other conditions that may trigger disease progression, treating it first before moving to other medications.[15] "[A]ntibiotic treatment for S. aureus infection leads to decreased erythroderma and tumor size without other adjunct treatment."[16][17]

Epidemiology

Mycosis fungoides is the most common form of cutaneous T-cell lymphoma.[2] In the western population there are around 0.3 cases of Sezary syndrome per 100,000 people.[2] Sézary disease is more common in males with a ratio of 2:1,[2] and the mean age of diagnosis is between 55 and 60 years of age.[2][6]

See also

References

  1. ↑ Sézary's cell at Who Named It?
  2. 1 2 3 4 5 6 7 8 9 10 11 12 Cuneo A, Castoldi. "Mycosis fungoidses/Sezary's syndrome". Retrieved 2008-02-15.
  3. ↑ 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. PMID 9129044.
  4. ↑ "Causes and Symptoms". Retrieved 2008-02-15.
  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. 1 2 Lorincz, A. I. "Sezary syndrome". Retrieved 2008-02-15.
  7. 1 2 3 "Diagnosis". Retrieved 2008-02-15.
  8. ↑ Beigi, Pooya Khan Mohammad (2017). Clinician's Guide to Mycosis Fungoides. Springer International Publishing. pp. 13–18. ISBN 9783319479064. doi:10.1007/978-3-319-47907-1_4.
  9. ↑ 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. PMID 24438970. doi:10.1016/j.jaad.2013.08.033.
  10. ↑ 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.
  11. ↑ 2016 Chicago 2-Day: Research Update https://www.youtube.com/watch?v=3GB6LV7GrsI
  12. ↑ Williams SC. "The rarest of rashes". Stanford Medicine.
  13. ↑ 2016 Los Angeles PEF - Integrative Medicine and Cutaneous Lymphoma https://www.youtube.com/watch?v=koi4ZRSDrtQ
  14. ↑ Novel Therapies for CTCL - Andrei Shustov, MD https://www.youtube.com/watch?v=OTYvjH_YuY8
  15. ↑ Skin bacteria may fuel cancer cell progression - March 02, 2016 - By Lisette Hilton http://dermatologytimes.modernmedicine.com/dermatology-times/news/skin-bacteria-may-fuel-cancer-cell-progression
  16. ↑ INFECTIOUS COMPLICATIONS OF CUTANEOUS T-CELL LYMPHOMA, March/April 2001 https://moffitt.org/File%20Library/Main%20Nav/Research%20and%20Clinical%20Trials/Cancer%20Control%20Journal/v8n2/185.pdf
  17. ↑ Association of Erythrodermic Cutaneous T-Cell Lymphoma, Superantigen-Positive Staphylococcus aureus, and Oligoclonal T-Cell Receptor Vβ Gene Expansion, Blood 1997, http://www.bloodjournal.org/content/89/1/32?sso-checked=true
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