Mycosis fungoides overview

Cutaneous T cell lymphoma Microchapters
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Overview
Classification Mycosis fungoides Sezary syndrome
Pathophysiology

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. 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. 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 psoriasis. 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. 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 findings of cutaneous T cell lymphoma include fever, rash, pruritus, ulcer, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy. Laboratory tests for cutaneous T cell lymphoma include complete blood count (CBC), blood chemistry studies, flow cytometry, immunohistochemistry, and immunophenotyping. 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. 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.

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-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

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. 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.

Risk Factors

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

Natural History, Complications 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.

Diagnosis

Staging

The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.

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.

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.

Laboratory tests

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

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.

MRI

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

Other Imaging Studies

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

Other Diagnostic Studies

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

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.

References

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