Mikozis (mycosis) fungoides

 

mikozis fungoides

 

When we talk about lymphomas, which are a type of cancer of the body's lymph tissues, it is interesting to know that there is a type of lymphoma that originates in a type of white blood cell located in the skin called type T lymphocytes. Cutaneous T-cell lymphoma is a collection of lymphomas that all originate from cutaneous T-lymphocytes but with slight differences. The most common type of cutaneous T-cell lymphoma is Mycosis Fungoides. In this article we will talk about skin manifestations, clinical signs, causes, stages and treatment of 'Mycosis fungoides'.

 

How does mycosis fungoides form?

Types of T lymphocytes in our skin

Causes of mycosis fungoides disease

People prone to mycosis fungoides

Prevalence of mycosis fungoides disease

Stages of the mycosis fungoides disease

Symptoms of mycosis fungoides

Differential diagnosis of mycosis fungoides and cesarean section

How is mycosis fungoides diagnosed?

What factors play a role in the prognosis of mycosis fungoides disease?

How is mycosis fungoides treated?

 

How does mycosis fungoides form?

Our skin is the body's first line of defense against the entry of pathogens. Immune system has its soldiers ready in our skin. These soldiers are type T lymphocytes (lymphocytes are a type of white blood cell that has two types of B and T). Unfortunately, these T lymphocytes can themselves become cancerous. 

 

Types of T lymphocytes in our skins

Normally, two types of T lymphocytes live in our skin and protect the body against pathogens.

  • T-lymphocyte (CD4+) called T-helper
  • T lymphocyte (CD45ro+) called T-cell memory

During mycosis fungoides, there are uncontrolled proliferation of T-cell (both memory and helper T-cells) called cancer cell colonies, which often lack normal T cells that have antigens of CD2, CD5, and CD7 (for more about CD read here). Mycosis fungoides is sometimes called "MF disease".

 

Causes of mycosis fungoides disease

There are two theories for the development of mycosis fungoides disease. In the first theory, continuous presence of some antigens and foreign agents in the skin is leading to the development of cancer. On the other hand, second theory believes that some viruses whose antigens cause genetic changes in T cells is the reason behind the development of cancer cells.

For whatever reason it is, groups of cancerous T cells called colonies develop in this disease. MF disease can also be a rare type of non-Hodgkin's lymphoma that starts from skin but can affect other organs throughout the course of the disease. Unfortunately, the exact cause of mycosis fungoides disease is still unknown.

 

People prone to mycosis fungoides:

Most people with mycosis fungoides have one or more abnormal chromosomal and genetic conditions. For example, some of the genes have been lost or substances have been added to their genetics. These genetic changes found in cancer cells are usually developed over many years of a person's life.

 

Prevalence of Mycosis Fungoides disease:

This disease is more common in men than women. It is mostly seen in people over forty-five years old. People with special occupations such as glassmakers, people who make pottery and ceramics are more likely to develop this disease.

 

Stages of mycosis fungoides disease:

The disease has several stages in terms of clinical manifestations:

  1. Patch stage
  2. Plaque stage
  3. Tumor stage

It usually takes months to years to move from one stage to the next one. If diagnosed in the early days, they are often treatable.

 

Symptoms of mycosis fungoides:

Main symptoms include:

  • Dry and itchy skin
  • The appearance of dark spots on the surface of the skin
  • Red and scaly skin blemishes
  • Prominent and rough skin lesions
  • Cutaneous nodules
  • Persistent and severe itching

If we want to describe the symptoms of this disease from start to end, respectively, we must classify them in the following order:

Premycotic phase:

Red, scaly rash that often occurs in areas of the body that are not exposed to sunlight. This scaly red lesion may remain asymptomatic for months to years, so it is difficult to diagnose mycosis fungoides at this stage.

Patch phase:

The skin lesions are thin, red and eczema-like.

Plaque phase:

Small, prominent lesions like the papule appear. Then red and hardened skin lesions appear on the surface of the skin.

Tumor phase:

In this stage, tumor-like lesions form on the surface of the skin that can become infected.

 

Differential diagnosis of mycosis fungoides disease and sezary syndromes:

Sezary syndrome is another type of cutaneous T-cell lymphoma. In this disease, the entire skin of the body becomes red and itchy, which can be accompanied by skin peeling and pain. Sezary syndrome in addition to having all the signs and symptoms of MF disease, have symptoms like general redness of the skin which accompanied with pain and itching. In Sezary syndrome cancerous T lymphocytes are present in the bloodstream.

Some physicians consider sezary syndrome to be an advanced form of mycosis fungoides disease or mycosis fungoides leukemia lymphoma. Another group considers Sezary syndrome as an independent non-Hodgkin's lymphoma.  

 

How is mycosis fungoides diagnosed?

  • Clinical examination and observation of skin lesions along with accurate sampling of suspected skin lesions
  • Immunophenotyping studies on tissue samples (division of cells by the characteristics of proteins in the cell wall)
  • Flow cytometry (measuring the dimensions of a cell)
  • T cell receptor re-arrangement test (T cell receptor test)
  • Blood tests to study changes in blood cells

After diagnosing mycosis fungoides, the doctor will order various tests, including PET scans and CT scans. These imaging techniques will determine if organs and internal parts of the body are also involved or not. Lymph node biopsies as well as bone marrow biopsies and aspirations are also used along with imaging techniques.

 

What factors play a role in the prognosis of mycosis fungoides disease?

Both the prognosis (what the patient will experience in the future), the choice of treatment and patient's response to treatments depend on the following factors:

  • The stage at which a patient is diagnosed (There are 4 stage for this disease)
  • Type of skin lesions (patch, plaque or tumor)
  • Age of the patient
  • Gender of the patient

Mycosis fungoides and sezary syndrome are very difficult to treat. Most treatments used are palliative therapies to reduce symptoms and improve the patient's life. Patients diagnosed at a very early stage can survive for years.

 

Mycosis fungoides treatments

Treatment options for patients with mycosis fungoides disease include:

  • Photochemotherapy (radiation therapy)
  • Topical steroids
  • Short periods of UV treatment (in autumn and winter)
  • A topical drug called Nitrogen Mustard (Mechlorethamine)
  • Interferons
  • Oral retinoid drugs
  • Photopheresis (patient's blood is taken, changed, then re-injected)
  • Antifolate: methotrexate or pralatrexate
  • Interferon
  • Alemtuzumab (campath)
  • Brentuximab
  • Mogamulizumab
  • Chemotherapy drugs (chemotherapy)
  • Gemcitabine
  • Doxil
  • Bone marrow transplant

 

 

 

 

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