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Mycosis fungoides

MedGen UID:
7771
Concept ID:
C0026948
Neoplastic Process
Synonyms: Granuloma fungoides
Modes of inheritance:
Multifactorial inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Sources: HPO, Orphanet
A mode of inheritance that depends on a mixture of major and minor genetic determinants possibly together with environmental factors. Diseases inherited in this manner are termed complex diseases.
not inherited
MedGen UID:
832438
Concept ID:
CN227390
Intellectual Product
Source: Orphanet
Describes a disorder that is not inherited.
not inherited (Orphanet)
SNOMED CT: MF - Mycosis fungoides (118618005); Mycosis fungoides (90120004); Mycosis fungoides (clinical) (118618005); Mycosis fungoides (118618005)
 
OMIM®: 254400
Orphanet: ORPHA2584

Definition

Mycosis fungoides is a malignant T-cell lymphoma of the skin, first reported (and named) by Alibert (1835). Sezary syndrome is a leukemic variant of mycosis fungoides defined by erythroderma with greater than 80% of the skin showing redness, adenopathy and greater than 1,000 circulating Sezary cells/microliter with a CD4+CD26- or CD4+CD7- phenotype. Sezary cells have a type 2 helper T cell cytokine profile. Sezary syndrome has a median overall survival time of only 2.4 years in patients with Sezary cells at a density of greater than 10,000 cells/microliter or 5.4 years in patients with 1,000-10,000 Sezary cells/microliter. Mycosis fungoides and Sezary syndrome are the most common cutaneous T-cell lymphomas. Sezary syndrome can arise de novo or can appear following years of chronic mycosis fungoides. Both are thought to arise from clonal expansion of CD4+ helper T cells responding to chronic antigen stimulation (summary by Wang et al., 2015). [from GTR]

Additional descriptions

From OMIM
Mycosis fungoides is a malignant T-cell lymphoma of the skin, first reported (and named) by Alibert (1835). Sezary syndrome is a leukemic variant of mycosis fungoides defined by erythroderma with greater than 80% of the skin showing redness, adenopathy and greater than 1,000 circulating Sezary cells/microliter with a CD4+CD26- or CD4+CD7- phenotype. Sezary cells have a type 2 helper T cell cytokine profile. Sezary syndrome has a median overall survival time of only 2.4 years in patients with Sezary cells at a density of greater than 10,000 cells/microliter or 5.4 years in patients with 1,000-10,000 Sezary cells/microliter. Mycosis fungoides and Sezary syndrome are the most common cutaneous T-cell lymphomas. Sezary syndrome can arise de novo or can appear following years of chronic mycosis fungoides. Both are thought to arise from clonal expansion of CD4+ helper T cells responding to chronic antigen stimulation (summary by Wang et al., 2015).  http://www.omim.org/entry/254400
From GHR
Mycosis fungoides is the most common form of a type of blood cancer called cutaneous T-cell lymphoma. Cutaneous T-cell lymphomas occur when certain white blood cells, called T cells, become cancerous; these cancers characteristically affect the skin, causing different types of skin lesions. Although the skin is involved, the skin cells themselves are not cancerous. Mycosis fungoides usually occurs in adults over age 50, although affected children have been identified.Mycosis fungoides may progress slowly through several stages, although not all people with the condition progress through all stages. Most affected individuals initially develop skin lesions called patches, which are flat, scaly, pink or red areas on the skin that can be itchy. Cancerous T cells, which cause the formation of patches, are found in these lesions. The skin cells themselves are not cancerous; the skin problems result when cancerous T cells move from the blood into the skin. Patches are most commonly found on the lower abdomen, upper thighs, buttocks, and breasts. They can disappear and reappear or remain stable over time. In some affected individuals, patches progress to plaques, the next stage of mycosis fungoides.Plaques are raised lesions that are usually reddish, purplish, or brownish in color and itchy. Plaques commonly occur in the same body regions as patches. While some plaques arise from patches, others develop on their own, and an affected person can have both patches and plaques simultaneously. As with patches, cancerous T cells are found in plaques. Plaques can remain stable or can develop into tumors. Not everyone with patches or plaques develops tumors.The tumors in mycosis fungoides, which are composed of cancerous T cells, are raised nodules that are thicker and deeper than plaques. They can arise from patches or plaques or occur on their own. Mycosis fungoides was so named because the tumors can resemble mushrooms, a type of fungus. Common locations for tumor development include the upper thighs and groin, breasts, armpits, and the crook of the elbow. Open sores may develop on the tumors, often leading to infection.Although rare, the cancerous T cells can spread to other organs, including the lymph nodes, spleen, liver, and lungs. Spread to other organs can occur in any stage of mycosis fungoides but is most common in the tumor stage. In addition, affected individuals have an increased risk of developing another lymphoma or other type of cancer.  https://ghr.nlm.nih.gov/condition/mycosis-fungoides

Clinical features

Neoplasm of the skin
MedGen UID:
40101
Concept ID:
C0007114
Neoplastic Process
A primary or metastatic tumor involving the skin. Primary malignant skin tumors most often are carcinomas (either basal cell or squamous cell carcinomas that arise from cells in the epidermis) or melanomas that arise from pigment-containing skin melanocytes. Metastatic tumors to the skin include carcinomas and lymphomas.
Lymphoma
MedGen UID:
44223
Concept ID:
C0024299
Neoplastic Process
A cancer originating in lymphocytes and presenting as a solid tumor of lymhpoid cells.
Erythema
MedGen UID:
11999
Concept ID:
C0041834
Disease or Syndrome
Red discoloration of the skin caused by infectious agents, inflammation, drug hypersensitivity, or underlying disease.
Lymphoma
MedGen UID:
44223
Concept ID:
C0024299
Neoplastic Process
A cancer originating in lymphocytes and presenting as a solid tumor of lymhpoid cells.
Eczematous rash
MedGen UID:
3968
Concept ID:
C0013595
Disease or Syndrome
A form of dermatitis characterized by red, itchy, scaly, or crusty patches that can be chronic or intermittent.(NICHD)
Lymphadenopathy
MedGen UID:
96929
Concept ID:
C0497156
Disease or Syndrome
An enlarged lymph node. Causes include viral and bacterial infections and cancers that affect the lymph nodes.
Neoplasm of the skin
MedGen UID:
40101
Concept ID:
C0007114
Neoplastic Process
A primary or metastatic tumor involving the skin. Primary malignant skin tumors most often are carcinomas (either basal cell or squamous cell carcinomas that arise from cells in the epidermis) or melanomas that arise from pigment-containing skin melanocytes. Metastatic tumors to the skin include carcinomas and lymphomas.
Eczematous rash
MedGen UID:
3968
Concept ID:
C0013595
Disease or Syndrome
A form of dermatitis characterized by red, itchy, scaly, or crusty patches that can be chronic or intermittent.(NICHD)
Pruritus
MedGen UID:
19534
Concept ID:
C0033774
Sign or Symptom
A skin disorder characterized by an intense itching sensation.
Erythema
MedGen UID:
11999
Concept ID:
C0041834
Disease or Syndrome
Red discoloration of the skin caused by infectious agents, inflammation, drug hypersensitivity, or underlying disease.
Skin plaque
MedGen UID:
69134
Concept ID:
C0241148
Finding
A large (greater than 5-10 mm) raised skin lesion with a wide, flat surface.(NICHD)

Recent clinical studies

Etiology

Cengiz FP, Emiroglu N, Ozkaya DB, Bahali AG, Su O, Onsun N
Ann Clin Lab Sci 2017 Jan;47(1):25-28. PMID: 28249912
Hughes CF, Newland K, McCormack C, Lade S, Prince HM
Australas J Dermatol 2016 Aug;57(3):182-91. Epub 2015 May 18 doi: 10.1111/ajd.12349. PMID: 25988337
Brown DN, Wieser I, Wang C, Dabaja BS, Duvic M
J Am Acad Dermatol 2015 Dec;73(6):976-86. Epub 2015 Oct 21 doi: 10.1016/j.jaad.2015.09.017. PMID: 26476898
Cengiz FP, Emiroglu N
An Bras Dermatol 2015 Jan-Feb;90(1):36-40. doi: 10.1590/abd1806-4841.20153352. PMID: 25672297Free PMC Article
Marschalkó M, Erős N, Kontár O, Hidvégi B, Telek J, Hársing J, Jókai H, Bottlik G, Rajnai H, Szepesi Á, Matolcsy A, Kárpáti S, Csomor J
J Eur Acad Dermatol Venereol 2015 May;29(5):964-72. Epub 2014 Nov 18 doi: 10.1111/jdv.12743. PMID: 25406034

Diagnosis

Magro CM, Nguyen GH
Indian J Dermatol Venereol Leprol 2016 Jul-Aug;82(4):395-403. doi: 10.4103/0378-6323.181502. PMID: 27279297
Ceriolo P, Fausti V, Cinotti E, Bonadio S, Raffaghello L, Bianchi G, Orcioni GF, Fiocca R, Rongioletti F, Pistoia V, Borgonovo G
World J Gastroenterol 2016 Mar 28;22(12):3496-501. doi: 10.3748/wjg.v22.i12.3496. PMID: 27022231Free PMC Article
Hughes CF, Newland K, McCormack C, Lade S, Prince HM
Australas J Dermatol 2016 Aug;57(3):182-91. Epub 2015 May 18 doi: 10.1111/ajd.12349. PMID: 25988337
Brown DN, Wieser I, Wang C, Dabaja BS, Duvic M
J Am Acad Dermatol 2015 Dec;73(6):976-86. Epub 2015 Oct 21 doi: 10.1016/j.jaad.2015.09.017. PMID: 26476898
Kim J, Foster R, Lam M, Kumarasinghe SP
Australas J Dermatol 2015 Feb;56(1):49-51. Epub 2014 Feb 26 doi: 10.1111/ajd.12155. PMID: 24575903

Therapy

Foss FM, Girardi M
Hematol Oncol Clin North Am 2017 Apr;31(2):297-315. doi: 10.1016/j.hoc.2016.11.008. PMID: 28340880
Xue J, Liu C, Liu Y
Photodiagnosis Photodyn Ther 2017 Mar;17:87-91. Epub 2016 Nov 23 doi: 10.1016/j.pdpdt.2016.11.010. PMID: 27888162
Furudate S, Fujimura T, Kakizaki A, Hidaka T, Asano M, Aiba S
J Dermatol Sci 2016 Sep;83(3):182-9. Epub 2016 Jun 8 doi: 10.1016/j.jdermsci.2016.05.004. PMID: 27342040
Hughes CF, Newland K, McCormack C, Lade S, Prince HM
Australas J Dermatol 2016 Aug;57(3):182-91. Epub 2015 May 18 doi: 10.1111/ajd.12349. PMID: 25988337
Vandergriff T, Nezafati KA, Susa J, Karai L, Sanguinetti A, Hynan LS, Ambruzs JM, Oliver DH, Pandya AG
J Cutan Pathol 2015 May;42(5):318-28. Epub 2015 Apr 8 doi: 10.1111/cup.12470. PMID: 25721994

Prognosis

Jankowska-Konsur A, Kobierzycki C, Grzegrzółka J, Piotrowska A, Gomulkiewicz A, Glatzel-Plucinska N, Reich A, Podhorska-Okołów M, Dzięgiel P, Szepietowski JC
Acta Derm Venereol 2017 Feb 8;97(2):235-241. doi: 10.2340/00015555-2517. PMID: 27545193
Magro CM, Nguyen GH
Indian J Dermatol Venereol Leprol 2016 Jul-Aug;82(4):395-403. doi: 10.4103/0378-6323.181502. PMID: 27279297
Hughes CF, Newland K, McCormack C, Lade S, Prince HM
Australas J Dermatol 2016 Aug;57(3):182-91. Epub 2015 May 18 doi: 10.1111/ajd.12349. PMID: 25988337
Brown DN, Wieser I, Wang C, Dabaja BS, Duvic M
J Am Acad Dermatol 2015 Dec;73(6):976-86. Epub 2015 Oct 21 doi: 10.1016/j.jaad.2015.09.017. PMID: 26476898
Furue M, Kadono T
J Dermatol 2015 Oct;42(10):941-4. doi: 10.1111/1346-8138.13083. PMID: 26432429

Clinical prediction guides

Jankowska-Konsur A, Kobierzycki C, Grzegrzółka J, Piotrowska A, Gomulkiewicz A, Glatzel-Plucinska N, Reich A, Podhorska-Okołów M, Dzięgiel P, Szepietowski JC
Acta Derm Venereol 2017 Feb 8;97(2):235-241. doi: 10.2340/00015555-2517. PMID: 27545193
Ceriolo P, Fausti V, Cinotti E, Bonadio S, Raffaghello L, Bianchi G, Orcioni GF, Fiocca R, Rongioletti F, Pistoia V, Borgonovo G
World J Gastroenterol 2016 Mar 28;22(12):3496-501. doi: 10.3748/wjg.v22.i12.3496. PMID: 27022231Free PMC Article
Hughes CF, Newland K, McCormack C, Lade S, Prince HM
Australas J Dermatol 2016 Aug;57(3):182-91. Epub 2015 May 18 doi: 10.1111/ajd.12349. PMID: 25988337
LeBlanc RE, Lefterova MI, Suarez CJ, Tavallaee M, Kim YH, Schrijver I, Kim J, Gratzinger D
Hum Pathol 2015 Sep;46(9):1382-9. Epub 2015 Jun 10 doi: 10.1016/j.humpath.2015.05.024. PMID: 26193796
Marschalkó M, Erős N, Kontár O, Hidvégi B, Telek J, Hársing J, Jókai H, Bottlik G, Rajnai H, Szepesi Á, Matolcsy A, Kárpáti S, Csomor J
J Eur Acad Dermatol Venereol 2015 May;29(5):964-72. Epub 2014 Nov 18 doi: 10.1111/jdv.12743. PMID: 25406034

Recent systematic reviews

Xue J, Liu C, Liu Y
Photodiagnosis Photodyn Ther 2017 Mar;17:87-91. Epub 2016 Nov 23 doi: 10.1016/j.pdpdt.2016.11.010. PMID: 27888162
Brown DN, Wieser I, Wang C, Dabaja BS, Duvic M
J Am Acad Dermatol 2015 Dec;73(6):976-86. Epub 2015 Oct 21 doi: 10.1016/j.jaad.2015.09.017. PMID: 26476898
Dogra S, Mahajan R
Indian J Dermatol Venereol Leprol 2015 Mar-Apr;81(2):124-35. doi: 10.4103/0378-6323.152169. PMID: 25751327
Humme D, Nast A, Erdmann R, Vandersee S, Beyer M
Cancer Treat Rev 2014 Sep;40(8):927-33. Epub 2014 Jun 19 doi: 10.1016/j.ctrv.2014.06.005. PMID: 24997678
Weberschock T, Strametz R, Lorenz M, Röllig C, Bunch C, Bauer A, Schmitt J
Cochrane Database Syst Rev 2012 Sep 12;(9):CD008946. doi: 10.1002/14651858.CD008946.pub2. PMID: 22972128

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