Mycosis fungoides overview: Difference between revisions
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==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 lymphoma arises from [[T-cells]]. The [[malignant]] T cells in the body are pushed to the 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 plaques and [[tumor]]s before [[metastasis|metastatizing]] to other parts of the body. Based on the organ involvement, cutaneous T cell lymphoma may be classified into mycosis fungoides (MF) and sézary syndrome (SS).<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>Mycosis Fungoides was first described in 1806 by French dermatologist [[Jean-Louis-Marc Alibert]]. Sézary's disease was first described by Albert Sézary. 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 is caused by a mutation in the T cells. Cutaneous T cell lymphoma must be differentiated from other diseases such as [[eczema]] and [[psorasis]]. Mycosis fungoides commonly affects 45 and 55 years. [[Sézary syndrome]] commonly affects 60 years. 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> There are no established risk factors for cutaneous T cell lymphoma. 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> | '''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 lymphoma arises from [[T-cells]]. The [[malignant]] T cells in the body are pushed to the 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 plaques and [[tumor]]s before [[metastasis|metastatizing]] to other parts of the body. Based on the organ involvement, cutaneous T cell lymphoma may be classified into mycosis fungoides (MF) and sézary syndrome (SS).<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>Mycosis Fungoides was first described in 1806 by French dermatologist [[Jean-Louis-Marc Alibert]]. Sézary's disease was first described by Albert Sézary. 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 is caused by a mutation in the T cells. Cutaneous T cell lymphoma must be differentiated from other diseases such as [[eczema]] and [[psorasis]]. Mycosis fungoides commonly affects 45 and 55 years. [[Sézary syndrome]] commonly affects 60 years. 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> 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.<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> | ||
The staging of cutaneous T cell lymphoma is based on skin and lymph node 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 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> Common physical examination findings of cutaneous T cell lymphoma include [[fever]], [[rash]], [[pruritus]], [[ulcer]], chest tenderness, abdomen 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 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> Lymph node or skin biopsy is diagnostic of cutaneous T cell lymphoma. CT scan may be helpful in the diagnosis of cutaneous T cell lymphoma.<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> MRI may be helpful in the diagnosis of cutaneous T cell lymphoma.<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> [[PET]] scan may be helpful in the diagnosis of cutaneous T cell lymphoma.<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> Other diagnostic studies for cutaneous T cell lymphoma include [[bone marrow aspiration]] and [[bone marrow biopsy]]. <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 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 staging of cutaneous T cell lymphoma is based on skin and lymph node 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 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> Common physical examination findings of cutaneous T cell lymphoma include [[fever]], [[rash]], [[pruritus]], [[ulcer]], chest tenderness, abdomen 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 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> Lymph node or skin biopsy is diagnostic of cutaneous T cell lymphoma. CT scan may be helpful in the diagnosis of cutaneous T cell lymphoma.<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> MRI may be helpful in the diagnosis of cutaneous T cell lymphoma.<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> [[PET]] scan may be helpful in the diagnosis of cutaneous T cell lymphoma.<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> Other diagnostic studies for cutaneous T cell lymphoma include [[bone marrow aspiration]] and [[bone marrow biopsy]]. <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 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> | ||
==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. | ||
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==Risk Factors== | ==Risk Factors== | ||
There are no established risk factors for cutaneous T cell lymphoma. | There are no established risk factors for cutaneous T cell lymphoma. | ||
==Natural History and Prognosis== | |||
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. | |||
==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> | ||
Revision as of 14:18, 25 January 2016
|
Cutaneous T cell lymphoma Microchapters |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]
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 lymphoma arises from T-cells. The malignant T cells in the body are pushed to the surface of the skin in a biological process used to rid the body of offending material, causing various lesions 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 plaques and tumors before metastatizing to other parts of the body. Based on the organ involvement, cutaneous T cell lymphoma may be classified into mycosis fungoides (MF) and sézary syndrome (SS).[1]Mycosis Fungoides was first described in 1806 by French dermatologist Jean-Louis-Marc Alibert. Sézary's disease was first described by Albert Sézary. 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 is caused by a mutation in the T cells. Cutaneous T cell lymphoma must be differentiated from other diseases such as eczema and psorasis. Mycosis fungoides commonly affects 45 and 55 years. Sézary syndrome commonly affects 60 years. 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.[2] 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.[3] The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.[1]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] Common physical examination findings of cutaneous T cell lymphoma include fever, rash, pruritus, ulcer, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.[4] Laboratory tests for cutaneous T cell lymphoma include complete blood count (CBC), blood chemistry studies, flow cytometry, immunohistochemistry, and immunophenotyping.[4] Lymph node or skin biopsy is diagnostic of cutaneous T cell lymphoma. CT scan may be helpful in the diagnosis of cutaneous T cell lymphoma.[4] MRI may be helpful in the diagnosis of cutaneous T cell lymphoma.[4] PET scan may be helpful in the diagnosis of cutaneous T cell lymphoma.[4] Other diagnostic studies for cutaneous T cell lymphoma include bone marrow aspiration and bone marrow biopsy. [4]The predominant therapy for cutaneous T cell lymphoma is PUVA. Adjunctive chemotherapy, radiotherapy, biological therapy, retinoid therapy, and photophoresis may be required.[1]
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
Based on the organ involvement, cutaneous T cell lymphoma may be classified into mycosis fungoides (MF) and sézary syndrome (SS).[1]
Pathophysiology
Cutaneous T cell lymphoma arises from T-cells. 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
Cutaneous T cell lymphoma is caused by a mutation in the T cells.
Differential Diagnosis
Cutaneous T cell lymphoma must be differentiated from other diseases such as eczema and psorasis.
Epidemiology and demographics
Mycosis fungoides commonly affects 45 and 55 years. Sézary syndrome commonly affects 60 years. 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.[2]
Risk Factors
There are no established risk factors for cutaneous T cell lymphoma.
Natural History and Prognosis
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.
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.[5]
Diagnosis
Staging
The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.[1]
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, abdomen 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
Lymph node or skin biopsy is diagnostic of cutaneous T cell lymphoma.
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.[1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 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
- ↑ 2.0 2.1 Mycosis fungoides. Radiopaedia.http://radiopaedia.org/articles/mycosis-fungoides Accessed on January 21, 2016
- ↑ Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cutaneous+T+cell+lymphoma Accessed on January 19, 2016
- ↑ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 4.12 4.13 Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016
- ↑ Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=cutaneous+T+cell+lymphoma Accessed on January 19, 2016