×

Please Remove Adblock
Adverts are the main source of Revenue for DoveMed. Please remove adblock to help us create the best medical content found on the Internet.

Folliculotropic Mycosis Fungoides

Last updated Sept. 14, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Folliculotropic Mycosis Fungoides is a rare variant of Mycosis Fungoides, which is a cutaneous T-cell non-Hodgkin’s lymphoma. Here, the malignant lymphocytes involve the hair follicles, and hence, the name of the condition.


What are the other Names for this Condition? (Also known as/Synonyms)

  • Follicular Mycosis Fungoides
  • Folliculotropic MF
  • Pilotropic Mycosis Fungoides

What is Folliculotropic Mycosis Fungoides? (Definition/Background Information)

  • Folliculotropic Mycosis Fungoides is a rare variant of Mycosis Fungoides, which is a cutaneous T-cell non-Hodgkin’s lymphoma. Here, the malignant lymphocytes involve the hair follicles, and hence, the name of the condition
  • It is a form of primary cutaneous non-Hodgkin lymphoma, meaning it is a lymphoma that is first seen in the skin. At a later stage, it can involve other parts of the body including the lymph nodes and bone marrow. This is known as systemic Folliculotropic Mycosis Fungoides
  • Folliculotropic Mycosis Fungoides can result in the formation of skin lesions (patches and plaques) that may ulcerate and cause subsequent infections. If other parts of the body are also affected, widespread symptoms, such as weight loss, fatigue, anemia, fever, and night sweats, can also be present
  • Folliculotropic Mycosis Fungoides is usually diagnosed with a biopsy of the affected skin or taking a biopsy of the swollen lymph nodes or affected organ. Other tests and procedures may be used to examine the severity and extent of the condition
  • Skin-directed therapy or systemic therapy may be used to treat Folliculotropic Mycosis Fungoides. However, it has been noted in some patients that the tumor does not respond to treatment
  • The prognosis depends on many elements including the progression of the condition, response to treatment, and health status of the individual. In general, the prognosis is poor, since this variant is more aggressive than classical Mycosis Fungoides

General information on lymphoma and lymphocytes:

  • Lymphoma is a form of cancer stemming from uncontrollable division of lymphocytes (type of white blood cells). There are two types of lymphomas:
    • Hodgkin lymphoma
    • Non-Hodgkin lymphoma
  • Lymphocytes are the primary white blood cells located in the lymph, which is the fluid of the lymphatic system; just as blood is the fluid of the circulatory system
  • Lymphocytes are produced in bone marrow, and can mature into either B-cells or T-cells. T-cells mature in the thymus; Folliculotropic Mycosis Fungoides arises from cancerous T-cells
  • Lymph results from filtration of blood as it moves to and from tissues. Lymph is colorless because it contains no red blood cells; instead, it contains lymphocytes. It is central to the immune system
  • There are 3 different kinds of lymphocytes:
    • T-lymphocytes or T cells: They help fight infections and irregularities within the cells (cell-mediated immunity). They fight viruses and cancerous cells
    • B-lymphocytes or B cells: They generate antibodies that are bodily defense proteins, which target foreign invaders outside the cells (humoral immunity). They combat bacterial cells, cell fragments, and other immunogenic elements
    • Natural killer cells or NK cells: They perform various functions related to both cell-mediated and humoral immunity. They also search for cancer cells, a process called immune surveillance

Who gets Folliculotropic Mycosis Fungoides? (Age and Sex Distribution)

  • Folliculotropic Mycosis Fungoides is a rare disorder that generally affects adults in a wide age group
  • Both males and females can be affected
  • All races and ethnic groups can be affected

What are the Risk Factors for Folliculotropic Mycosis Fungoides? (Predisposing Factors)

Numerous studies have indicated the subsequent risk factors for Folliculotropic Mycosis Fungoides:

  • Cigarette smoking for 40 years or more
  • Skin conditions such as eczema
  • Family history of multiple myeloma
  • Individuals in professions such as painting, carpentry, farming, and pottery and ceramics
  • Viral infections

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Folliculotropic Mycosis Fungoides? (Etiology)

Lymphocytes are a type of white blood cells that are responsible for providing immunity in the human body. B-cells and T-cells are the two different types of lymphocytes. Under certain circumstances, when the lymphocytes grow and reproduce abnormally, it leads to a condition called lymphoma, which is a common type of cancer. There are 2 types of lymphoma:

  • Hodgkin lymphoma
  • Non-Hodgkin lymphoma

The cause for this type of non-Hodgkin lymphoma is unknown. Folliculotropic Mycosis Fungoides may be triggered by a viral infection. Also, there may be certain genetic defects, such as translocation, which is a juxtaposition of regions of the chromosomes. This may result in: 

  • Change in regulatory features of certain cancer-causing genes called oncogenes, which can lead to amplified production of their mRNA (overexpression), thus increasing their protein levels
  • Exchange of protein coding regions of genes, giving rise to new proteins that can encourage the inappropriate cell growth 

It is believed that the abnormal growth of lymphocytes gives rise to cancerous cells resulting in the formation of this condition. However, how this process occurs and the causative factors remain under investigation.

Note: Mycosis Fungoides is non-contagious and is not transmitted from one individual to another. One cannot contract the condition through close physical contact with the affected individual.

What are the Signs and Symptoms of Folliculotropic Mycosis Fungoides?

The signs and symptoms of Folliculotropic Mycosis Fungoides involving the skin include:

  • The skin patch occur anywhere in the body including in the head and neck region (most common site), unlike conventional Mycosis Fungoides, where the head and neck region is spared
  • The presence of a plaque or skin patch
  • The lesion involves the hair follicles, and hence, there is hair loss associated with the skin patch
  • The lesions can grow, ulcerate, and bleed

If the lymphoma expands to areas other than the skin, then the signs and symptoms depend on the organs involved. In such cases, the following general signs and symptoms of Folliculotropic Mycosis Fungoides may be present:

  • Unintentional weight loss; changes in appetite
  • Fatigue and weakness, headache
  • High temperatures and excessive night sweats (may be recurrent)
  • Anemia (low red blood cell count)
  • Low lymphocyte count, established by a blood test
  • Recurrent infections
  • Difficulty breathing
  • Low blood pressure
  • Back pain
  • Swelling of the legs
  • Abdominal pain and swelling; constipation
  • Frequent urination 

Folliculotropic Mycosis Fungoides can affect other parts of the body. Depending upon the specific organ or area involved, the signs and symptoms may include:

  • Painless swelling of lymph nodes in the neck, armpits, or groin
  • There may be associated autoimmune disorders, which can cause joint and muscle pain, heat intolerance, recurrent rashes, abdominal pain, and a general feeling of sickness
  • If the brain is involved, then the following neurological symptoms may be seen:
    • Confusion
    • Tinnitus (ringing in the ears)
    • Hearing and visual impairment
  • If the gastrointestinal (GI) tract is involved, then the following GI tract symptoms may be observed:
    • Ulcers
    • Diarrhea
    • Inflammation
    • GI bleeding
  • Liver and spleen enlargement
  • Joint inflammation and fluid buildup (edema) can occur, if the joints are affected

How is Folliculotropic Mycosis Fungoides Diagnosed?

Folliculotropic Mycosis Fungoides diagnosis is typically performed by obtaining biopsy samples from the affected region and examining them under a microscope to detect the cancerous cells. General methods to aid in the study-diagnosis of Folliculotropic MF include:

  • A thorough physical examination and a complete medical history
  • Blood tests that may include:
    • Complete blood cell count (CBC) blood test
    • Absolute lymphocyte count on peripheral blood
    • Liver function blood test (LFT)
    • Lactate dehydrogenase (LDH) blood test
  • Skin biopsy from the affected area:
    • A biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
    • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
    • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, flow cytometric analysis and very rarely, electron microscopic studies, to assist in the diagnosis
  • If the lymphoma includes the lymph nodes, biopsies of inflamed lymph nodes are taken and examined in a lab to determine if the cells are malignant or benign. The biopsies may be performed under general or local anesthesia. Normally, the whole lymph node is removed to help determine the subtype of lymphoma
  • Radiological imaging may be performed to determine the extent of lymphoma in the body including:
    • X-ray of the affected region
    • Ultrasound scan of the affected region
    • Computerized tomography (CT) scan of the affected region
    • Vascular radiological studies
    • Whole body bone scan
    • Whole body CT-PET scans to determine how much further the lymphoma has spread, by checking the size and metabolic rate (a reflection of uncontrolled growth) of lymph nodes, throughout the body. This can also help determine, if the cancer has spread to other organ systems
    • Brain MRIs are performed if neurological symptoms are present, which can help determine if the cancer has spread to the brain or to tissues that cover the brain
  • Bone marrow aspiration and biopsy is performed and sent to a laboratory for a pathological examination to determine if the bone marrow is involved. Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, histochemical stains, molecular testing, and very rarely electron microscopic studies. However, a bone marrow biopsy is not needed in the early stages of the condition
  • Flow cytometry to identify cells as they flow through an instrument, called a flow cytometer. Flow cytometry measures the quantity and percentage of cells in a blood sample, and cell characteristics such as size, shape, and the presence of biomarkers on the cell surface. This process helps to sub-classify the condition and also to detect remaining levels of disease after treatment. This tool can help in diagnosing relapse and can determine if restarting treatment is necessary
  • Fluorescence in situ hybridization (FISH): It is a test performed on the blood or bone marrow cells to identify chromosome changes (cytogenetic analysis) in blood cancer cells. The test helps in detecting genetic abnormalities that may not be obvious with an examination of cells under a microscope
  • Immunophenotyping to identify a specific kind of cell in a sample, which can help decide the best treatment course to follow
  • Polymerase chain reaction (PCR): It is used to measure the presence of certain biomarkers in blood or bone marrow cells. The test is ultrasensitive and detects extremely low amounts of biomarkers remaining in blood, which can be missed by cytogenetic methods, such as FISH, karyotype, or flow cytometry. PCR allows a more sensitive follow-up of patients in remission and can help determine whether additional treatment is necessary
  • Lumbar puncture to determine if the brain is involved
  • In addition, the cerebrospinal spinal fluid (CSF) may be collected by inserting a needle in the spine and subjected to microscopic, flow cytometric, PCR, and biochemical analysis, to diagnose central nervous system (CNS) involvement, if any
  • Exploratory laparoscopy (diagnostic laparoscopy) may be required, if gastrointestinal symptoms are present. In this procedure, the abdomen is examined using a minimally-invasive technique, and a tissue biopsy and tissue for culture obtained. Minimally-invasive approaches help decrease complications and the length of stay at the hospital. A diagnostic laparoscopy is also helpful in staging of the tumor. Nevertheless, this procedure is not very much used

Note: A differential diagnosis to eliminate other tumor types is often considered before arriving at a definitive diagnosis.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Folliculotropic Mycosis Fungoides?

The complications due to Folliculotropic Mycosis Fungoides may include the following:

  • Involvement of local and distant organs: It can spread from the lymph nodes to other parts of the body
  • Loss of function of the organ/area to which the cancer has spread
  • This condition is more aggressive than classical forms of Mycosis Fungoides
  • A weakened immune system (or immunosuppression) could be a complication of Folliculotropic Mycosis Fungoides, which could become more severe throughout treatment. Due to this, individuals are more vulnerable to infections; there is a heightened risk of developing serious complications from such infections
  • If the condition spreads to the brain and central nervous system, it can give rise to:
    • Inflammation of the meninges or brain (this can be lethal)
    • Headache
    • Vision changes
    • Facial numbness
  • If the abdomen is affected, it can cause intestinal obstruction that results in urine outflow obstruction and kidney damage
  • Occasionally, Folliculotropic Mycosis Fungoides can convert into a more destructive form of lymphoma

There may be complications related to chemotherapy used in treating the condition, which may include the following:

  • Side effects such as dizziness, vomiting, appetite loss, mouth ulcers, and hair loss
  • By damaging healthy cells, the individual is more vulnerable to subsequent infections
  • The treatment can also cause infertility in men and women. Hence, measures to protect the individual’s fertility must be considered before starting chemotherapy

The treatment methods can also give rise to secondary cancers such as skin cancer.

How is Folliculotropic Mycosis Fungoides Treated?

Once a definitive diagnosis of Folliculotropic Mycosis Fungoides has been made, staging is used to describe how far the cancer has spread. The staging can help describe:

  • How many lymph nodes are affected
  • Their locations in the body
  • And, if other organs are being affected

Staging is important because different treatment regimens are needed, depending on the progression of the lymphoma. The staging of Folliculotropic Mycosis Fungoides is as follows:

Stage I Folliculotropic Mycosis Fungoides: Presence of non-cancerous, but abnormal lymphocytes in blood.

  • Stage IA: The skin lesions (patches, papules, plaques) cover less than 10% of the body area
  • Stage IB: The skin lesions cover more than 10% of the body area

Stage II Folliculotropic Mycosis Fungoides: Presence of non-cancerous, but abnormal lymphocytes in blood.

  • Stage IIA: The skin lesions (patches, papules, plaques) can cover any percentage of the body area. The cancer has not affected the lymph nodes, but they are enlarged
  • Stage IIB: The presence of single or multiple tumors, over 1 cm in size (or larger), on the skin. The cancer has not affected the lymph nodes, but they may be enlarged

Stage III Folliculotropic Mycosis Fungoides:

  • Presence of non-cancerous, but abnormal lymphocytes in blood
  • The skin is red and the skin lesions (patches, papules, plaques) may be found almost on the entire body
  • The cancer has not affected the lymph nodes, but they may be enlarged

Stage IV Folliculotropic Mycosis Fungoides: The skin is red on most part of the body surface; the skin lesions (patches, papules, plaques) or tumors can cover any percentage of the body area.

  • Stage IVA: Additionally -
    • The cancer has affected the lymph nodes and there may be a presence of cancerous lymphocytes in blood, OR
    • The cancer has not affected the lymph nodes, but it may be enlarged; and there are cancerous lymphocytes in blood
  • Stage IVB: Additionally -
    • There is the involvement of other body organs
    • The cancer may have affected the lymph nodes, which may be enlarged
    • There may be the presence of cancerous lymphocytes in blood

The treatment measures depend upon the stage and location of the tumor. There are 2 main treatment protocols for Folliculotropic Mycosis Fungoides including:

  • Skin-directed therapy
  • Systemic therapy

Skin-directed therapies are employed (and often in combination) when the tumor is predominately confined to the skin. This may include the following treatment measures:

  • Sunlight therapy (or exposure to ultraviolet light)
  • Use of topical steroids and chemotherapy
  • Systemic chemotherapy
  • Radiation therapy to affected (local) skin area
  • Other systemic treatment methods, such as the use of interferons, retinoids, etc.
  • Biological therapy

Systemic therapy is generally used for advanced stage tumors. A combination of treatment methods may be used to effectively treat the condition, which may include the following:

  • Chemotherapy: This approach uses a combination of drugs to destroy the cancerous cells and can be used in patients of all stages of Folliculotropic Mycosis Fungoides
    • There can be severe side effects including fatigue, nausea, hair loss, anemia, high risk of infection, and drug-specific reactions
    • Many T-cell lymphomas can be resistant to chemotherapy. It can also damage healthy cells
    • Chemotherapy can be administered as a pill, liquid, shot, or intravenously

Note: Men and women in child-bearing age would greatly benefit from counseling regarding fertility issues. Some chemotherapy agents can cause infertility in both men and women. There can be permanent damage to the testicles and ovaries, harming their ability to produce sperms or ova. In men, sperm banking can be considered before initiating therapy. In women, in many cases, due to urgency of starting chemotherapy, it is often difficult to perform ovum banking. However, if there is sufficient time prior to chemotherapy, ovum banking may be performed. The healthcare provider may help assess the risk-benefit analysis, depending upon each individual’s specific circumstances.

  • Radiation: Radiation therapy is the use of high-energy radiation waves to kill cancer cells by destroying their DNA
    • This treatment modality is normally used for early stage lymphomas. It is most frequently used in combination with chemotherapy
    • The radiation may be administered by a machine placed outside the body, or by placing a radioactive material inside the body
    • The side effects of radiation therapy include nausea, vomiting, fatigue, pain, risk of cancer later in life, and risk of heart disease
    • Radiation can damage healthy cells in addition to cancer cells, causing further complications
  • Surgery: After initial biopsies to diagnose Folliculotropic Mycosis Fungoides, surgery is a very rare treatment option because lymphomas are systemic and might have already spread to more than one lymph node in most patients. Under such circumstances, removing all of the affected lymph nodes would be extremely difficult
  • Supportive treatment: Steroids, blood transfusions, anti-nausea medications, and antibiotics may be used as supportive therapy. In conjunction with other treatment measures, these can help combat the symptoms of immunodeficiency

If Folliculotropic Mycosis Fungoides is not fully responsive to treatment, or if the chance of recurrence is high, then bone marrow transplantation or stem cell transplantation can be considered.

  • Bone marrow transplantation: Characteristically, administering high doses of chemotherapy or radiotherapy may treat systemic cases of Folliculotropic MF. However, high doses of chemotherapy drugs will also cause harm to the bone marrow, preventing it from producing any blood cells. Hence, before starting high-dose chemotherapy, the healthcare provider may remove some of the patient’s bone marrow and freeze/preserve it. Collecting the bone marrow is called a bone marrow harvest. The bone marrow is then stored. After high dose chemotherapy or radiation, the bone marrow is thawed and inserted back into the patient through a drip (transfusion). This is called an autologous bone marrow transplant. Occasionally, bone marrow donated by a matching person (usually a brother or sister) is used, if the condition persists following transplant using one’s own cells. This is called as allogeneic bone marrow transplant
  • Stem cell transplantation: This procedure is similar to bone marrow transplantation and involves the replacement of healthy blood-forming stem cells into the body. The technique is also called hematopoietic progenitor cell transplantation. Stem cells can be collected from the bone marrow, circulating (peripheral) blood, and umbilical cord blood. It may either involve an autologous stem cell transplantation, where stem cells are harvested from individuals before treatment and transplanted back into the patient after treatment, or involve an allogeneic stem cell transplantation, where stem cells donated by another matching person (usually a brother or sister) is used, if the condition persists after stem cell transplant using one’s own cell. This is called as allogeneic stem cell transplant

Note: Allogeneic bone marrow and allogeneic stem cell transplants may have more side effects and complications, and this treatment may not be suitable for every individual. If allogeneic transplants come from a healthy donor with no malignant cells, then the chance of relapse may be lessened.

  • In order to prevent infections due to a weakened immune system caused by Folliculotropic Mycosis Fungoides or by its treatment, the patient is kept in an isolated ward and treated with suitable antibiotics
  • Nowadays, targeted therapies are being developed that can selectively destroy the cancer cells. Many of them are in the stage of clinical trials
  • Clinical trials: There may be some newer treatment options, currently on clinical trials, which can be considered for some patients depending on their respective risk factors

Your healthcare provider will determine the best course of treatment depending on your individual circumstances. In addition, follow-up care with routine screening and check-ups are imperative post-treatment.

How can Folliculotropic Mycosis Fungoides be Prevented?

Currently, the cause of Folliculotropic Mycosis Fungoides is unknown. Hence, there are no known methods to prevent tumor occurrence. Eliminating the variable risk factors may help in preventing the condition. The following factors may be considered:

  • Avoiding or stopping smoking
  • Undertaking appropriate and prompt treatment for skin conditions, such as eczema, or viral infections
  • Using appropriate protective apparel (face masks, hand gloves, protective clothing) and adhering to proper safety practices, especially if you are a painter, carpenter, farmer, or working in the ceramics industry
  • Healthy diet and exercise, as well as avoidance of unnecessary exposure to chemicals, may help lessen cancer risk
  • In order to avoid a relapse, or be prepared for a relapse, the entire diagnosis, treatment process, drugs administered, etc. should be well-documented and follow-up measures initiated

Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are required. Often several years of active vigilance are crucial and necessary.

What is the Prognosis of Folliculotropic Mycosis Fungoides? (Outcomes/Resolutions)

  • The prognosis of Folliculotropic Mycosis Fungoides depends upon a set of several factors, which include:
    • Stage of tumor: With lower-stage tumors, when the tumor is limited to the site of origin, the prognosis is usually excellent with suitable therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
    • Overall health status of the individual: Individuals with excellent health have a better prognosis than those with poor health
    • Age of the individual: Older individuals usually have a poorer prognosis than younger individuals
    • The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
    • Individuals with bulky disease have a worse prognosis
    • Involvement of vital organs may complicate the condition
    • The surgical respectability of the tumor (meaning, if the tumor can be removed completely) - it is a rare option
    • Whether the tumor is occurring for the first time, or is a recurring tumor. Recurring tumors have a poorer prognosis compared to tumors that do not recur
    • Response to treatment: Tumors that respond to treatment have a better prognosis compared to tumors that are unresponsive to treatment
    • Progression of the condition makes the outcome worse (progressive Folliculotropic Mycosis Fungoides)
  • An early diagnosis and prompt treatment of the tumor usually results in better outcomes than a late diagnosis and deferred treatment
  • The combination chemotherapy drugs used may have some severe side effects such as cardio-toxicity. This primarily impacts elderly adults, or those who are already affected by other medical conditions. Tolerance to chemotherapy sessions is a positive influencing factor
  • Progression to bone marrow failure is typically associated with short survival

Additional and Relevant Useful Information for Folliculotropic Mycosis Fungoides:

  • There are many variants of Mycosis Fungoides including:
    • Pagetoid Reticulosis
    • Syringotropic Mycosis Fungoides
    • Folliculotropic Mycosis Fungoides
    • Granulomatous Mycosis Fungoides
  • T-cell lymphomas are less common than B-cell lymphomas
  • Treatment for Folliculotropic Mycosis Fungoides can result in physical and emotional distress; supportive care and encouragement help positively and can bring a substantial amount of relief to the patient

The following article link will help you understand leukemia and lymphoma (blood cancer):

http://www.dovemed.com/diseases-conditions/leukemia-and-lymphoma/

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Feb. 16, 2016
Last updated: Sept. 14, 2018