mycosis fungoides/Sezary syndrome
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Etiology
- human T-lymphotrophic virus (HTLV) in some cases
Epidemiology
- mean age: 50 years; range 5-70 years
- male:female ratio 27:1
- uncommon, but NOT rare
Pathology
- bandlike & patchy infiltrate of atypical lymphocytes (Lutzner cells) in upper dermis extending to skin appendages
- microabscesses in the epidermis containing Lutzner cells
- small cells with cerebriform nuclei
- mycosis fungoides is the cutaneous form, Sezary syndrome is the leukemic form
Immunophenotype
Genetics
- T-cell receptor TCR genes clonally rearranged
- associated with defects in NAV3 gene
- overexpression of cornifelin
- other aberrantly expressed genes: ZMYND8
Clinical manifestations
- onset of lesions: months to years
- pruritus: often intractable, but may be none
- types of skin lesions
- maculopapular eruptions or plaques > 3 cm in size
- nodules or tumors with or without ulceration
- poikiloderma with or without plaques & nodules
- diffuse erythema
- color of skin lesions: different shades of red
- shape of skin lesions
- round, oval , arciform, annular, concentric, bizarre
- arrangement/distribution of skin lesions
- randomly distributed discrete lesions
- diffuse involvement with erythroderma (Sezary syndrome) 'red man syndrome'
- lesions occur on both sun-exposed & sun-protected skin[4]
- often spares exposed regions in early stages
- lymphadenopathy (Sezary syndrome)
- hair loss (Sezary syndrome)
* images[8] (folliculotropic mycosis fungoides)
Laboratory
- complete blood count (CBC)
- leukocytosis
- eosinophilia 6-16%; may be as high as 50%
- buffy coat, abnormal circulating T-cells (Sezary cells)
- bone marrow aspirate/biopsy NOT helpful
- serum chemistries
- serum lactate dehydrogenase (isozyme 1-3) increased in erythrodermic phase (isozyme analysis requires LDH electrophoresis)
- skin biopsy
- lymph node biopsy if palpable
- immunophenotyping
- autoantibodies against PARD3, CEP290
- see ARUP consult[5]
Radiology
- chest X-ray for hilar adenopathy
- computed tomography (CT) of the abdomen for retroperitoneal lymph nodes
- liver-spleen scan for focal areas of involvement
Complications
- increased risk of second malignancy (lymphoma, others)
Differential diagnosis
- psoriasis
- nummular eczema
- poikiloderma atrophicans vasculare
- cutaneous B cell lymphoma
- leukemia cutis
Management
- pharmaceutical agents
- 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
- disease unresponsive to topical glucocorticoids
- stage 3 & 4
- topical nitrogen mustard (10%) alone or in combination with electron beam therapy
- electron beam therapy plus systemic chemotherapy
- extracorporeal photopheresis
- histone deacetylase inhibitor
- alemtuzumab
- early stage disease (stage 1 & 2) limited to the skin is treated with topical glucocorticoids[4]
- 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
- stage 3 & 4 with extensive skin & organ involvement
- poor prognostic indicators
More general terms
Additional terms
- 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
References
- ↑ Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 544-49
- ↑ WHO Classification Tumours of Haematopoietic and Lymphoid Tissues. IARC Press 2001
- ↑ 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://pubmed.ncbi.nlm.nih.gov/17224541
- ↑ 4.0 4.1 4.2 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 18. American College of Physicians, Philadelphia 2009, 2012, 2018.
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ 5.0 5.1 ARUP Consult: Sezary Syndrome The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/sezary-syndrome
- ↑ Galper SL, Smith BD, Wilson LD. Diagnosis and management of mycosis fungoides. Oncology (Williston Park). 2010 May;24(6):491-501. PMID: https://pubmed.ncbi.nlm.nih.gov/20568590
- ↑ Prince HM, Whittaker S, Hoppe RT. How I treat mycosis fungoides and Sezary syndrome. Blood. 2009 Nov 12;114(20):4337-53 PMID: https://pubmed.ncbi.nlm.nih.gov/19696197
- ↑ 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://pubmed.ncbi.nlm.nih.gov/3510756 https://jamanetwork.com/journals/jamadermatology/fullarticle/2788259
- ↑ Khan N, Noor SJ, Horwitz S. How we treat mycosis fungoides and Sezary syndrome. Clin Adv Hematol Oncol. 2021;19:573-81. PMID: https://pubmed.ncbi.nlm.nih.gov/34495021
- ↑ Mycosis Fungoides and the Sezary Syndrome (PDQ): Treatment http://www.nci.nih.gov/cancertopics/pdq/treatment/mycosisfungoides/HealthProfessional