Mycosis fungoides overview

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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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