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mycosis fungoides/Sezary syndrome

Etiology: - human T-lymphotrophic virus (HTLV) in some cases Epidemiology: 1) mean age: 50 years; range 5-70 years 2) male:female ratio 27:1 3) uncommon, but NOT rare Pathology: 1) bandlike & patchy infiltrate of atypical lymphocytes (Lutzner cells) in upper dermis extending to skin appendages 2) microabscesses in the epidermis containing Lutzner cells 3) small cells with cerebriform nuclei 4) mycosis fungoides is the cutaneous form, Sezary syndrome is the leukemic form Immunophenotype: - CD2+, CD3+, CD5+, - ~1/3 CD7+, - most CD4+, - rare CD8+ cases - usually CD25-, DHRS8+ Genetics: - T-cell receptor TCR genes clonally rearranged - associated with defects in NAV3 gene - overexpression of cornifelin - other aberrantly expressed genes: ZMYND8 Clinical manifestations: 1) onset of lesions: months to years 2) pruritus: often intractable, but may be none 3) types of skin lesions a) maculopappular eruptions or plaques > 3 cm in size b) nodules or tumors with or without ulceration c) poikiloderma with or without plaques & nodules d) diffuse erythema 4) color of skin lesions: different shades of red 5) shape of skin lesions - round, oval , arciform, annular, concentric, bizarre 6) arrangement/distribution of skin lesions a) randomly distributed discrete lesions b) diffuse involvement with erythroderma (Sezary syndrome) 'red man syndrome' c) often spares exposed regions in early stages 7) lymphadenopathy (Sezary syndrome) 8) hair loss (Sezary syndrome) * images [8] (folliculotropic mycosis fungoides) Laboratory: 1) complete blood count (CBC) a) leukocytosis b) eosinophilia 6-16%; may be as high as 50% 2) buffy coat, abnormal circulating T-cells (Sezary cells) 3) bone marrow aspirate/biopsy NOT helpful 4) serum chemistries - serum lactate dehydrogenase (isozyme 1-3) increased in erythrodermic phase (isozyme analysis requires LDH electrophoresis) 5) skin biopsy 6) lymph node biopsy if palpable 7) immunophenotyping 8) autoantibodies against PARD3, CEP290 9) see ARUP consult [5] Radiology: 1) chest X-ray for hilar adenopathy 2) computed tomography (CT) of the abdomen for retroperitoneal lymph nodes 3) liver-spleen scan for focal areas of involvement Differential diagnosis: 1) psoriasis 2) nummular eczema 3) poikiloderma atrophicans vasculare 4) cutaneous B cell lymphoma 5) leukemia cutis Complications: - increased risk of second malignancy (lymphoma, others) Management: 1) pharmaceutical agents a) early stage disease (stage 1 & 2) limited to the skin is treated with topical glucocorticoids [4] - disease unresponsive to topical glucocorticoids - add retinoid such as bexarotene - PUVA therapy b) stage 3 & 4 - topical nitrogen mustard (10%) alone or in combination with electron beam therapy - electron beam therapy plus systemic chemotherapy - CHOP - extracorporeal photopheresis - histone deacetylase inhibitor - romidepsin, vorinostat - alemtuzumab 2) prognosis: - poor prognostic indicators - tumors present - lymphadenopathy - > 10% of surface area of skin involved - generalized erythroderma - age > 60 years - early stage disease (stage 1 & 2) limited to the skin - median survival > 20 years - stage 3 & 4 with extensive skin & organ involvement - median survival 4 years - mean survival at time of diagnosis: 5 years in USA

Interactions

disease interactions

Related

cutaneous B-cell lymphoma leukemia cutis Lutzner cell (mycosis cell) mechlorethamine; nitrogen mustard (Mustargen, Nor-mustard, Chlorethazine) nummular (discoid) eczema; exudative dermatitis oral psoralen & ultraviolet (UV) A light (PUVA) poikiloderma psoriasis Sezary cell T cell receptor

General

cutaneous T-cell lymphoma (CTCL)

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: 17224541
  4. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 18. American College of Physicians, Philadelphia 2009, 2012, 2018.
  5. 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: 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: 19696197
  8. Gu AK, Shi J, Zhang Y. Images in Dermatology. Folliculotropic Mycosis Fungoides. JAMA Dermatol. Published online February 2, 2022 PMID: 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