mycosis fungoides/Sezary syndrome

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Etiology

Epidemiology

  • mean age: 50 years; range 5-70 years
  • male:female ratio 27:1
  • uncommon, but NOT rare

Pathology

Immunophenotype

  • CD2+, CD3+, CD5+,
  • ~1/3 CD7+,
  • most CD4+,
  • rare CD8+ cases
  • usually CD25-, DHRS8+

Genetics

Clinical manifestations

* images[8] (folliculotropic mycosis fungoides)

Laboratory

Radiology

Complications

Differential diagnosis

Management

More general terms

Additional terms

References

  1. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 544-49
  2. WHO Classification Tumours of Haematopoietic and Lymphoid Tissues. IARC Press 2001
  3. Huang KP et al, Second lymphomas and other malignant neoplasms in patients with mycosis fungoides and Sezary syndrome: Evidence from population- based and clinical cohorts. Arch Dermatol 2007, 143:45 PMID: https://www.ncbi.nlm.nih.gov/pubmed/17224541
  4. 4.0 4.1 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 18. American College of Physicians, Philadelphia 2009, 2012, 2018.
  5. 5.0 5.1 ARUP Consult: Sezary Syndrome The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/sezary-syndrome
  6. Galper SL, Smith BD, Wilson LD. Diagnosis and management of mycosis fungoides. Oncology (Williston Park). 2010 May;24(6):491-501. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20568590
  7. Prince HM, Whittaker S, Hoppe RT. How I treat mycosis fungoides and Sezary syndrome. Blood. 2009 Nov 12;114(20):4337-53 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19696197
  8. 8.0 8.1 Gu AK, Shi J, Zhang Y. Images in Dermatology. Folliculotropic Mycosis Fungoides. JAMA Dermatol. Published online February 2, 2022 PMID: https://www.ncbi.nlm.nih.gov/pubmed/3510756 https://jamanetwork.com/journals/jamadermatology/fullarticle/2788259
  9. Mycosis Fungoides and the Sezary Syndrome (PDQ): Treatment http://www.nci.nih.gov/cancertopics/pdq/treatment/mycosisfungoides/HealthProfessional

Patient information

mycosis fungoides/Sezary syndrome patient information