Қол терісінің Т-жасушалы лимфомасы: Микоз фунгоид

Most common form of cutaneous T cell lymphoma

Mycosis fungoides, also known as Alibert Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesions, and itchy skin. While the cause remains unclear, most cases are not hereditary. Most cases are in people over 20 years of age, and it is more common in men than women. Treatment options include sunlight exposure, ultraviolet light, topical corticosteroids, chemotherapy, and radiotherapy.

Mycosis fungoides was first described in 1806 by French dermatologist Jean Louis Marc Alibert. The name mycosis fungoides is very misleading—it loosely means "mushroom like fungal disease". The disease, however, is not a fungal infection but rather a type of non Hodgkin's lymphoma. It was so named because Alibert described the skin tumors of a severe case as having a mushroom like appearance.

The symptoms of mycosis fungoides are categorized into three clinical stages: the patch stage, the plaque stage, and the tumour stage. The patch stage is defined by flat, reddish patches of varying sizes that may have a wrinkled appearance. They can also look yellowish in people with darker skin. It is characterized by the presence of raised lesions that appear reddish brown; in darker skin tones, plaques may have a greyish or silver appearance. Both patch and plaque stages are considered early stage mycosis fungoides. The symptoms displayed are progressive, with early stages consisting of lesions presented as scaly patches. Lesions often initially develop on the trunk of the body in places that are rarely exposed to the sun, such as the buttocks. Hypopigmentation (when the skin is lighter than normal) of lesions are less common but can be found in children, adolescents and/or dark skinned individuals. The advanced stage of mycosis fungoides is characterized by generalized erythroderma (red rash covering most of the body) with severe pruritus (itching) and scaling. Those that experience intense pruritus commonly indicate that it negatively affects their quality of life emotionally, functionally and physically. Mycosis fungoides (MF) and Sézary syndrome (SS) are related conditions, with the same type of cancer T lymphocytes, that initially grow in different body compartments. SS cells are found mainly in the blood, whereas MF typically involves the skin. In advanced stages of MF, the cancer cells move from the skin into other organs and the bloodstream; this progression is referred to as "leukemic mycosis fungoides", "Sézary syndrome preceded by mycosis fungoides", or "secondary mycosis fungoides".

Mycosis fungoides is caused by abnormal white blood cells (T lymphocytes). These abnormal cells have a preference for localizing and proliferating uncontrolled in the outer layer of the skin (epidermis). The abnormal cells may later involve other organs such as the lymph nodes. It is hypothesized that the genetic mutations in these cancer cells lead to increased growth and escape from programmed cell death. Additionally, the disease is an unusual expression of CD4 T cells, a part of the immune system. These T cells are skin associated, meaning they are biochemically and biologically most related to the skin, in a dynamic manner. Mycosis fungoides is the most common type of cutaneous T cell lymphoma (CTCL), but there are many other types of CTCL that have nothing to do with mycosis fungoides and these disorders are treated differently.

Traditionally, mycosis fungoides has been divided into three stages: premycotic, mycotic and tumorous. The premycotic stage clinically presents as an erythematous (red), itchy, scaly lesion. Microscopic appearance is non diagnostic and represented by chronic nonspecific dermatosis associated with psoriasiform changes in epidermis. In the mycotic stage, infiltrative plaques appear and biopsy shows a polymorphous inflammatory infiltrate in the dermis that contains small numbers of frankly atypical lymphoid cells. These cells may line up individually along the epidermal basal layer. The latter finding if unaccompanied by spongiosis is highly suggestive of mycosis fungoides. In the tumorous stage a dense infiltrate of medium sized lymphocytes with cerebriform nuclei expands the dermis. Accurate staging of mycosis fungoides is essential to determine appropriate treatment and prognosis. Staging is based on the tumor, node, metastasis, blood (TNMB) classification proposed by the Mycosis Fungoides Cooperative Group and revised by the International Society for Cutaneous Lymphomas/European Organization of Research and Treatment of Cancer. Systemic treatments of mycosis fungoides often lead to resistance; as such, additional treatment options are often necessary in advanced disease. Other treatments have been suggested, however, larger and more extensive research is needed to identify effective treatment strategies for this disease. Mogamulizumab is a CCR4 monoclonal antibody which has been shown to improve progression free survival. It was approved by the US FDA in 2018 for use in people with relapsed or refractory mycosis fungoides or Sézary disease. Narrowband UV B is commonly considered for children, as opposed to Psoralen with UV A, mechlorethamine hydrochloride, or oral bexarotene, which is often used in adults. The complete remission rate in children is nearly 30%. The incidence of mycosis fungoides was seen to be increasing between 2000 and 2020, although certain regions have demonstrated some stabilization.

In 1995 actor Mr. T was diagnosed with a cutaneous T cell lymphoma, or mycosis fungoides. Once in remission, he joked about the coincidence: "Can you imagine that? Cancer with my name on it — personalized cancer!" Popular British television actor Paul Eddington died of mycosis fungoides after living with the condition for four decades.