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{{Cutaneous T cell lymphoma}}
{{Mycosis fungoides}}
{{CMG}}; {{AE}} {{S.G.}}
{{CMG}}; {{AE}} {{S.G.}}


==Overview==
==Overview==
'''Cutaneous T-Cell lymphoma''' (CTCL) is a class of [[non-Hodgkin's lymphoma]], which is a type of [[cancer]] of the [[immune system]]. Cutaneous T-cell [[Lymphomas|lymphoma]] (CTCL) is infiltration of malignant T cells and activated T cells  in the skin. Cutaneous T cell lymphoma arises from [[T-cell]] lymphocytes, which are normally involved in the [[Cell (biology)|cell]] mediated [[immune]] response. The [[malignant]] T cells in the [[body]] are pushed to the [[Surface area|surface]] of the [[skin]] in a [[biological]] process used to rid the [[body]] of offending material, causing various [[lesion]]s to appear on the [[skin]]. These [[lesions]] change shape as the [[disease]] progresses, typically beginning as what appears to be a [[rash]] and eventually forming [[Plaque|plaques]] and [[tumor]]s before [[metastasis|metastatizing]] to other parts of the [[Human body|body]]. There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes. Mycosis Fungoides was first described in 1806 by French dermatologist [[Jean-Louis-Marc Alibert]]. Sezary's disease was first described by Albert Sézary. On [[microscopic]] [[histopathological]] [[analysis]],  atypical [[lymphoid]] [[Cell (biology)|cells]],  [[polymorphous]] [[inflammatory]] infiltrate in the dermis, and [[lymphocytes]] with cerebroid [[nuclei]] are characteristic findings of mycosis fungoides. Cutaneous T cell lymphoma is caused by a [[mutation]] in the T cells. Cutaneous T cell lymphoma must be differentiated from other diseases such as  [[eczema]] and [[psoriasis]]. Mycosis fungoides commonly affects  45 and 55  years. [[Sézary syndrome]] commonly affects 60 years. In the United States, [[Male|males]] are more commonly affected with cutaneous T cell lymphoma than [[Female|females]]. In the United States, cutaneous T cell lymphoma usually affects individuals of the African American [[race]].There are no established risk factors for cutaneous T cell lymphoma. If left untreated, cutaneous T cell lymphoma may progress to develop patches , plaque, and tumors. Depending on the extent of the lymphoma at the time of diagnosis, the prognosis may vary. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cutaneous T cell lymphoma.The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.The most common symptoms of cutaneous T cell lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]], [[itching]], chest pain, [[abdominal pain]], and [[bone pain]]. Common [[Physical examination|physical]] [[Physical examination|examination]] findings of cutaneous T cell lymphoma include [[fever]], [[rash]], [[pruritus]], [[ulcer]], [[chest]] [[Tenderness (medicine)|tenderness]], [[Abdomen|abdominal]] [[Tenderness (medicine)|tenderness]], [[bone]] [[tenderness]], [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].[[Medical laboratory|Laboratory]] tests for cutaneous T cell lymphoma include [[complete blood count]] ([[CBC]]), blood chemistry studies, [[flow cytometry]], [[immunohistochemistry]],  and [[immunophenotyping]]. The definitive diagnosis of cutaneous T cell lymphoma  is confirmed by either a [[skin]] [[biopsy]] or a [[lymph node]] [[biopsy]]. CT scan may be helpful in the diagnosis of cutaneous T cell lymphoma. MRI may be helpful in the diagnosis of cutaneous T cell lymphoma. [[PET]] scan may be helpful in the diagnosis of cutaneous T cell lymphoma.Other diagnostic studies for cutaneous T cell lymphoma include [[bone marrow aspiration]] and [[bone marrow biopsy]].The predominant therapy for cutaneous T cell lymphoma is [[PUVA]]. Adjunctive [[chemotherapy]], [[radiotherapy]], [[biological therapy]], retinoid therapy, and photophoresis may be required.
Mycosis fungoides is rare lymphomas of CD4+ helper T cells. Mycosis Fungoides was first described in 1806 by French dermatologist [[Jean-Louis-Marc Alibert]]. On [[microscopic]] [[histopathological]] [[analysis]],  atypical [[lymphoid]] [[Cell (biology)|cells]],  [[polymorphous]] [[inflammatory]] infiltrate in the dermis, and [[lymphocytes]] with cerebroid [[nuclei]] are characteristic findings of mycosis fungoides. Mycosis fungoides is caused by a [[mutation]] in the T cells. Mycosis fungoides must be differentiated from other [[Disease|diseases]] such as  [[eczema]] and [[psoriasis]]. Mycosis fungoides commonly affects  45 and 55  years. In the United States, [[Male|males]] are more commonly affected with Mycosis fungoides than [[Female|females]]. In the United States, Mycosis fungoides usually affects individuals of the African American [[race]].The [[Risk factor|risk factors]] in the development of mycosis fungoides is environmental, [[Occupational safety and health|occupational]]<nowiki/>exposure to long term exposure to [[chemicals]], virainfection. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for cutaneous T cell lymphoma.The staging of mycosis fungoides is based on skin and lymph node involvement.The most common symptoms of mycosis fungoides include [[fever]], [[weight loss]], [[skin]] [[rash]], [[night sweats]], [[itching]], [[Chest pain|chest pain,]] [[abdominal pain]], and [[bone pain]]. Common [[Physical examination|physical]] [[Physical examination|examination]] findings of mycosis fungoides include [[fever]], [[rash]], [[pruritus]], [[ulcer]], [[chest]] [[Tenderness (medicine)|tenderness]], [[Abdomen|abdominal]] [[Tenderness (medicine)|tenderness]], [[bone]] [[tenderness]], [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].[[Medical laboratory|Laboratory]] tests for mycosis fungoides include [[complete blood count]] ([[CBC]]), [[blood]] [[chemistry]] studies, [[flow cytometry]], [[immunohistochemistry]],  and [[immunophenotyping]]. The definitive diagnosis of mycosis fungoides is confirmed by either a [[skin]] [[biopsy]] or a [[lymph node]] [[biopsy]]. [[CT scanning]] in mycosis fungoides patients evaluated information such as enlarged [[lymph node]] (LN) and [[metastatic]] involvement. MRI may be helpful in the diagnosis of mycosis fungoides. [[PET]] scan may be helpful in the [[diagnosis]] of mycosis fungoides.Other [[diagnostic]] studies for mycosis fungoides include [[bone marrow aspiration]] and [[bone marrow biopsy]].The predominant [[therapy]] for mycosis fungoides is [[PUVA]]. Adjunctive [[chemotherapy]], [[radiotherapy]], [[biological therapy]], [[retinoid]] [[therapy]], and photophoresis may be required.
 
==Historical Perspective==
==Historical Perspective==
Mycosis Fungoides was first described in 1806 by French dermatologist [[Jean-Louis-Marc Alibert]]. Sézary's disease was first described by Albert Sézary  
Mycosis Fungoides was first described in 1806 by French dermatologist [[Jean-Louis-Marc Alibert]]. Sézary's [[disease]] was first described by Albert Sézary  
==Classification==
==Classification==
There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes.
There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes.
==Pathophysiology==
==Pathophysiology==
Cutaneous T cell lymphoma arises from [[T-cell]] lymphocytes, which are normally involved in the cell mediated immune response. On microscopic histopathological analysis,  atypical [[lymphoid]] cells,  polymorphous inflammatory infiltrate in the dermis, and [[lymphocytes]] with cerebroid nuclei are characteristic findings of mycosis fungoides.
Cutaneous T cell lymphoma arises from [[T-cell]] lymphocytes, which are normally involved in the cell mediated [[Immunity (medical)|immune]] response. On [[microscopic]] [[histopathological]] [[analysis]],  atypical [[lymphoid]] [[Cell (biology)|cells]],  polymorphous [[Inflammation|inflammatory]] infiltrate in the [[dermis]], and [[lymphocytes]] with cerebroid [[nuclei]] are characteristic findings of mycosis fungoides.
==Causes==
==Causes==
Development of cutaneous T cell lymphoma is the result of multiple genetic mutations.
Development of cutaneous T cell lymphoma is the result of multiple [[genetic mutations]].


==Differential Diagnosis==
==Differential Diagnosis==
Cutaneous T cell lymphoma must be differentiated from other diseases such as  [[eczema]] and [[psoriasis]].
Cutaneous T cell lymphoma must be differentiated from other diseases such as  [[eczema]] and [[psoriasis]].
==Epidemiology and Demographics==
==Epidemiology and Demographics==
The incidence of mycosis fungoides increases with age; the median age at diagnosis is between 45 and 55 years of age. The median age at diagnosis of Sézary syndrome is between 60 years of age. In the United States, males are more commonly affected with cutaneous T cell lymphoma than females. In the United States, cutaneous T cell lymphoma usually affects individuals of the African American race.<ref name="radio">Mycosis fungoides. Radiopaedia.http://radiopaedia.org/articles/mycosis-fungoides Accessed on January 21, 2016</ref>
The incidence of mycosis fungoides increases with [[age]]; the median age at [[diagnosis]] is between 45 and 55 years of age. The median age at [[diagnosis]] of Sézary syndrome is between 60 years of age. In the United States, [[Male|males]] are more commonly affected with cutaneous T cell lymphoma than [[Female|females]]. In the United States, cutaneous T cell lymphoma usually affects individuals of the African American [[race]].<ref name="radio">Mycosis fungoides. Radiopaedia.http://radiopaedia.org/articles/mycosis-fungoides Accessed on January 21, 2016</ref>
==Risk Factors==
==Risk Factors==
There are no established risk factors for cutaneous T cell lymphoma.
There are no established [[Risk factor|risk factors]] for cutaneous T cell lymphoma.
==Screening==
==Screening==
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cutaneous T cell lymphoma.<ref>Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cutaneous+T+cell+lymphoma Accessed on January 19, 2016</ref>
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for [[cutaneous]] T cell lymphoma.<ref>Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cutaneous+T+cell+lymphoma Accessed on January 19, 2016</ref>
==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
If left untreated, cutaneous T cell lymphoma may progress to develop cutaneous patches and plaque. Depending on the extent of the lymphoma at the time of diagnosis, the prognosis may vary.
If left untreated, cutaneous T cell lymphoma may progress to develop cutaneous patches and plaque. Depending on the extent of the [[Lymphomas|lymphoma]] at the time of [[diagnosis]], the [[prognosis]] may vary.
==Diagnosis==
==Diagnosis==
===Staging===
===Staging===
The staging of cutaneous T cell lymphoma is based on the skin and lymph nodes involvement.<ref name="canadiancancer">Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016</ref>
The staging of cutaneous T cell lymphoma is based on the [[skin]] and [[Lymph node|lymph nodes]] involvement.<ref name="canadiancancer">Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016</ref>


===Symptoms===
===Symptoms===
The most common symptoms of cutaneous T cell lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]], [[itching]], chest pain, [[abdominal pain]], and [[bone pain]].<ref name="seer.cancer">Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016</ref>
The most common [[Symptom|symptoms]] of cutaneous T cell lymphoma include [[fever]], [[weight loss]], [[skin]] [[rash]], [[night sweats]], [[itching]], [[chest]] [[pain]], [[abdominal pain]], and [[bone pain]].<ref name="seer.cancer">Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016</ref>
===Physical Examination===
===Physical Examination===
Common physical examination findings of cutaneous T cell lymphoma include [[fever]], [[rash]], [[pruritus]], [[ulcer]], chest tenderness, abdominal tenderness, bone tenderness, [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].<ref name="seer.cancer">Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016</ref>
Common physical examination findings of cutaneous T cell lymphoma include [[fever]], [[rash]], [[pruritus]], [[ulcer]], [[chest]] [[Tenderness (medicine)|tenderness]], [[Abdomen|abdominal]] [[tenderness]], [[bone]] [[tenderness]], [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].<ref name="seer.cancer">Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016</ref>


===Laboratory Tests===
===Laboratory Tests===
Laboratory tests for cutaneous T cell lymphoma include [[complete blood count]] (CBC), blood chemistry studies, [[flow cytometry]], [[immunohistochemistry]],  and [[immunophenotyping]].<ref name="seer.cancer">Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016</ref>
[[Laboratory]] tests for cutaneous T cell lymphoma include [[complete blood count]] (CBC), [[blood]] [[chemistry]] studies, [[flow cytometry]], [[immunohistochemistry]],  and [[immunophenotyping]].<ref name="seer.cancer">Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016</ref>
===Biopsy===
===Biopsy===
The definitive diagnosis of cutaneous T cell lymphoma  is confirmed by either a skin biopsy or a lymph node biopsy.
The definitive diagnosis of cutaneous T cell lymphoma  is confirmed by either a [[skin]] [[biopsy]] or a [[lymph node]] [[biopsy]].


===CT===
===CT===
Line 48: Line 49:
==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
The predominant therapy for cutaneous T cell lymphoma is [[PUVA]]. Adjunctive [[chemotherapy]], [[radiotherapy]], [[biological therapy]], retinoid therapy, and photophoresis may be required.<ref name="canadiancancer">Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016</ref>
The predominant [[therapy]] for cutaneous T cell lymphoma is [[PUVA]]. Adjunctive [[chemotherapy]], [[radiotherapy]], [[biological therapy]], retinoid therapy, and photophoresis may be required.<ref name="canadiancancer">Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016</ref>




==References==
==References==
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{{reflist|2}}
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Latest revision as of 22:50, 29 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

Mycosis fungoides is rare lymphomas of CD4+ helper T cells. Mycosis Fungoides was first described in 1806 by French dermatologist Jean-Louis-Marc Alibert. On microscopic histopathological analysis, atypical lymphoid cells, polymorphous inflammatory infiltrate in the dermis, and lymphocytes with cerebroid nuclei are characteristic findings of mycosis fungoides. Mycosis fungoides is caused by a mutation in the T cells. Mycosis fungoides must be differentiated from other diseases such as eczema and psoriasis. Mycosis fungoides commonly affects 45 and 55 years. In the United States, males are more commonly affected with Mycosis fungoides than females. In the United States, Mycosis fungoides usually affects individuals of the African American race.The risk factors in the development of mycosis fungoides is environmental, occupationalexposure to long term exposure to chemicals, virainfection. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cutaneous T cell lymphoma.The staging of mycosis fungoides is based on skin and lymph node involvement.The most common symptoms of mycosis fungoides include fever, weight loss, skin rash, night sweats, itching, chest pain, abdominal pain, and bone pain. Common physical examination findings of mycosis fungoides include fever, rash, pruritus, ulcer, chest tenderness, abdominal tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.Laboratory tests for mycosis fungoides include complete blood count (CBC), blood chemistry studies, flow cytometry, immunohistochemistry, and immunophenotyping. The definitive diagnosis of mycosis fungoides is confirmed by either a skin biopsy or a lymph node biopsy. CT scanning in mycosis fungoides patients evaluated information such as enlarged lymph node (LN) and metastatic involvement. MRI may be helpful in the diagnosis of mycosis fungoides. PET scan may be helpful in the diagnosis of mycosis fungoides.Other diagnostic studies for mycosis fungoides include bone marrow aspiration and bone marrow biopsy.The predominant therapy for mycosis fungoides is PUVA. Adjunctive chemotherapy, radiotherapy, biological therapy, retinoid therapy, and photophoresis may be required.

Historical Perspective

Mycosis Fungoides was first described in 1806 by French dermatologist Jean-Louis-Marc Alibert. Sézary's disease was first described by Albert Sézary

Classification

There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes.

Pathophysiology

Cutaneous T cell lymphoma arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response. On microscopic histopathological analysis, atypical lymphoid cells, polymorphous inflammatory infiltrate in the dermis, and lymphocytes with cerebroid nuclei are characteristic findings of mycosis fungoides.

Causes

Development of cutaneous T cell lymphoma is the result of multiple genetic mutations.

Differential Diagnosis

Cutaneous T cell lymphoma must be differentiated from other diseases such as eczema and psoriasis.

Epidemiology and Demographics

The incidence of mycosis fungoides increases with age; the median age at diagnosis is between 45 and 55 years of age. The median age at diagnosis of Sézary syndrome is between 60 years of age. In the United States, males are more commonly affected with cutaneous T cell lymphoma than females. In the United States, cutaneous T cell lymphoma usually affects individuals of the African American race.[1]

Risk Factors

There are no established risk factors for cutaneous T cell lymphoma.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cutaneous T cell lymphoma.[2]

Natural History, Complications and Prognosis

If left untreated, cutaneous T cell lymphoma may progress to develop cutaneous patches and plaque. Depending on the extent of the lymphoma at the time of diagnosis, the prognosis may vary.

Diagnosis

Staging

The staging of cutaneous T cell lymphoma is based on the skin and lymph nodes involvement.[3]

Symptoms

The most common symptoms of cutaneous T cell lymphoma include fever, weight loss, skin rash, night sweats, itching, chest pain, abdominal pain, and bone pain.[4]

Physical Examination

Common physical examination findings of cutaneous T cell lymphoma include fever, rash, pruritus, ulcer, chest tenderness, abdominal tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.[4]

Laboratory Tests

Laboratory tests for cutaneous T cell lymphoma include complete blood count (CBC), blood chemistry studies, flow cytometry, immunohistochemistry, and immunophenotyping.[4]

Biopsy

The definitive diagnosis of cutaneous T cell lymphoma is confirmed by either a skin biopsy or a lymph node biopsy.

CT

CT scan may be helpful in the diagnosis of cutaneous T cell lymphoma.[4]

MRI

MRI may be helpful in the diagnosis of cutaneous T cell lymphoma.[4]

Other Imaging Studies

PET scan may be helpful in the diagnosis of cutaneous T cell lymphoma.[4]

Other Diagnostic Studies

Other diagnostic studies for cutaneous T cell lymphoma include bone marrow aspiration and bone marrow biopsy. [4]

Treatment

Medical Therapy

The predominant therapy for cutaneous T cell lymphoma is PUVA. Adjunctive chemotherapy, radiotherapy, biological therapy, retinoid therapy, and photophoresis may be required.[3]


References

  1. Mycosis fungoides. Radiopaedia.http://radiopaedia.org/articles/mycosis-fungoides Accessed on January 21, 2016
  2. Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cutaneous+T+cell+lymphoma Accessed on January 19, 2016
  3. 3.0 3.1 Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016


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