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

• Syringotropic MF
• Syringotropic Mycosis Fungoides with Alopecia

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

• Syringotropic Mycosis Fungoides is a rare variant of Mycosis Fungoides, which is a cutaneous T-cell non-Hodgkin’s lymphoma. It is usually present as a single, well-defined plaque and can affect the eccrine sweat glands
• Since, there is an associated hair loss with the plaque, the condition is also commonly known as Syringotropic Mycosis Fungoides with Alopecia
• Syringotropic MF is a type of primary cutaneous non-Hodgkin lymphoma that has its origins in the skin. When the disease spreads to other parts of the body including the lymph nodes and bone marrow, is it known as systemic Syringotropic Mycosis Fungoides
• Syringotropic MF could manifest itself as skin lesions (papules and plaques), which may ulcerate and cause secondary infections. When the disease spreads, generalized symptoms, such as weight loss, fatigue, anemia, fever, and night sweats may be present
• The gold standard of diagnosis of Syringotropic Mycosis Fungoides is biopsy of skin, swollen lymph nodes, or affected organ. Other exams and tests may be conducted to assess spread of the condition
• The stage of Syringotropic Mycosis Fungoides at diagnosis determines treatment options. For example, for localized Syringotropic MF, a skin-directed treatment (such as the use of topical medications, phototherapy, or local radiation therapy) may be recommended. On the other hand, for advanced stages, a systemic therapy (through a combination of chemotherapy, radiation therapy, and surgery) may be prescribed
• Many factors contribute to the ultimate outcome of the condition. These include stage at diagnosis, progression of the condition, response to treatment, age and overall health of the patient. In general, the prognosis is considered to be good when the diagnosis is early, followed by prompt and appropriate treatment

General information on lymphoma and lymphocytes:

• Lymphoma is a type of cancer stemming from erratic, unregulated proliferation of lymphocytes (type of white blood cells). There are two types of lymphomas:
  • Hodgkin lymphoma
  • Non-Hodgkin lymphoma
• Lymphocytes are the predominant white blood cells found in the lymph, the fluid of the lymphatic system (similar to blood in the circulatory system)
• Lymph results from filtration of blood as it travels to and from tissues. Lymph is colorless because it lacks red blood cells; instead, it contains lymphocytes. It is central to the immune system
• Lymphocytes are generated in the bone marrow, and are of 3 different kinds of lymphocytes:
  • T-lymphocytes or T cells: These cells mature in the thymus and help combat infections and abnormalities within the cells (cell-mediated immunity). They fight viruses and cancerous cells. Syringotropic Mycosis Fungoides has its origins in cancerous T-cells
  • B-lymphocytes or B cells: They produce antibodies that are bodily defense proteins, which target foreign invaders outside the cells (humoral immunity). They fight bacterial cells, cell fragments, and other immunogenic elements
  • Natural killer cells or NK cells: They perform diverse functions related to both cell-mediated and humoral immunity. They also scout for cancer cells, a process called immune surveillance

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

• Syringotropic Mycosis Fungoides is a rare disorder that generally affects adults in the age group 25-85 years
• Both genders are reported to be affected by the disease
• No racial or ethnic predilection has been observed in the occurrence of Syringotropic MF

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

Scientific research has identified the following risk factors for Syringotropic Mycosis Fungoides:

• Cigarette smoking for 40 years or more
• Skin conditions such as eczema
• Family history of multiple myeloma
• Occupations 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 Syringotropic Mycosis Fungoides? (Etiology)

Lymphocytes are the predominant type of white blood cells in the lymph and provide immunity in the human body. There are different types of lymphocytes. Under certain circumstances, the lymphocytes grow and multiply abnormally, leading to a condition called lymphoma, a most common type of cancer. There are 2 types of lymphoma:

• Hodgkin lymphoma
• Non-Hodgkin lymphoma

Syringotropic Mycosis Fungoides is a non-Hodgkin lymphoma, whose causative factors are unknown. Some scientists theorize that the disease may be caused by a viral infection. Additionally, certain genetic defects, such as a translocation, are believed to cause Syringotropic MF as well. A translocation occurs when dissimilar chromosomes (Example: One chromosome 12 and one chromosome 22) are juxtaposed, and there is exchange of parts between the two. The translocation could lead to:

• Re-arrangement of regulatory elements of certain cancer-causing genes (oncogenes). This could lead to increased production of their mRNA (overexpression), instead of suppression of oncogene expression
• Exchange of protein coding regions of gene, giving rise to novel proteins that can stimulate the aberrant cell proliferation

It is believed that the abnormal development of lymphocytes gives rise to cancerous cells leading to the formation of this condition. Nevertheless, how this occurs and the factors that cause it remain under investigation.

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

What are the Signs and Symptoms of Syringotropic Mycosis Fungoides?

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

• The presence of a usually well-defined single plaque
• There is hair loss associated with the plaque
• The affected area can have increased/decreased pigmentation
• Some individuals may complain of loss of sensation over the skin patches
• Any skin part on the body may be affected; common locations include the chest, back, arms and legs
• The eccrine sweat glands are generally affected
• The lesions can grow, ulcerate and spread to other parts of the body

The skin is the organ primarily affected by Syringotropic Mycosis Fungoides, with symptoms such as plaques and papules. If other organs are affected, some general symptoms might ensue, such as the following:

• 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
• Frequent infections
• Trouble breathing
• Low blood pressure
• Back pain
• Swelling of the legs
• Abdominal pain and swelling; constipation
• Frequent urination 

Apart from the above general symptoms, specific symptoms pertaining to the organ affected could be observed as well. 

• Non-painful swelling of lymph nodes in the neck, armpits, or groin
• Associated autoimmune disorders, causing
  • Joint and muscle pain
  • Inability to tolerate heat
  • Recurrent rashes
  • Abdominal pain
  • General malaise
• Brain
  • Confusion
  • Tinnitus (ringing in the ears)
  • Hearing and visual impairment
• Gastrointestinal (GI) tract
  • Ulcers
  • Diarrhea
  • Inflammation
  • GI bleeding
• Enlargement of organs including liver and spleen
• Joints
  • Inflammation
  • Edema (fluid accumulation)

How is Syringotropic Mycosis Fungoides Diagnosed?

Syringotropic Mycosis Fungoides is typically diagnosed by biopsy. Additional tests may be required to identify and localize disease spread, if any. Tests used for the diagnosis of the condition may include: 

• A thorough physical examination and evaluation of personal and family medical history
• Blood tests:
  • Complete blood cell count (CBC) blood test
  • Absolute lymphocyte count on peripheral blood
  • Liver function blood test (LFT)
  • Lactate dehydrogenase (LDH) blood test
• Biopsy of skin from the affected area:
  • Tissue removed from the tumor is 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 the 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, special studies (immunohistochemical stains, molecular testing, flow cytometric analysis and very rarely, electron microscopic studies, etc.) may be required to assist in the diagnosis
• If the lymphoma involves the lymph nodes then, biopsies of enlarged lymph nodes are conducted. The lymph node may be excised from the patient under general or local anesthesia. Normally, the entire lymph node is removed to help determine the subtype of lymphoma
• Radiological imaging of an affected region may be needed to determine the extent of spread of lymphoma in the body. The following radiological examinations may be undertaken:
  • X-ray
  • Ultrasound scan
  • Computerized tomography (CT) scan of the affected region
  • Vascular radiological studies
  • Whole body bone scan
  • Whole body CT-PET scans - to determine how far 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 used 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 may be performed and sent to a laboratory for a pathological examination, to determine if the bone marrow has the cancer. Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, histochemical stains, molecular testing, and very rarely electron microscopic studies. It must be noted that a bone marrow biopsy is generally not deemed necessary 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 number 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 method helps to sub-classify the condition and also to detect residual levels of disease after treatment. This tool can help in diagnosing relapse and restart treatment as needed
• Fluorescence in situ hybridization (FISH): It is a test performed on the blood or bone marrow cells to detect chromosome changes (cytogenetic analysis) in blood cancer cells. The test helps in identifying genetic abnormalities that may not be evident with an examination of cells under a microscope
• Immunophenotyping to identify a specific type of cell in a sample, which can help determine the best treatment course to be followed
• 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: Differential diagnoses, to eliminate other tumor types are 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 Syringotropic Mycosis Fungoides?

Potential complications due to Syringotropic Mycosis Fungoides may include: 

• Spread of cancer from lymph nodes to other regions and organs
• Loss of function of the organ/area to which cancer has spread
• Vulnerability to infections: Immunosuppression as a result of lymph nodes being affected could become more severe during treatment, leaving the patient vulnerable to infections and serious complications
• Brain and central nervous system involvement (if they are affected by the cancer) leading to:
  • Inflammation of the meninges or brain (potentially fatal)
  • Headaches
  • Vision changes
  • Facial numbness
• Intestinal obstruction, urine outflow obstruction, and kidney damage, owing to cancer spreading to the abdominal region
• Transformation of Syringotropic MF to a more aggressive form of lymphoma

Additionally, treatment methods, such as chemotherapy, could lead to certain undesirable side effects, such as:

• Dizziness, vomiting, appetite loss, mouth ulcers, and hair loss
• Damage to healthy cells, leaving the patient susceptible to secondary infections
• Possibility of infertility in men and women (hence, measures to protect the individual’s fertility must be considered, before starting chemotherapy)

Note: Some treatment measures could potentially give rise to secondary cancers, such as skin cancer.

How is Syringotropic Mycosis Fungoides Treated?

Once a definitive diagnosis of Syringotropic Mycosis Fungoides has been made, staging is used to determine if the cancer is localized or systemic. The staging can help describe:

• The number and location/s of lymph nodes are affected
• Organs where the cancer has spread to

Staging provides an insight into the lymphoma and its spread, if any, and treatment regimens are based on staging. The following are the various stages of Syringotropic Mycosis Fungoides:

Stage I Syringotropic 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 Syringotropic 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 Syringotropic 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 Syringotropic 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

There are 2 main treatment protocols for Syringotropic Mycosis Fungoides:

• kin-directed therapy
• Systemic therapy

When the tumor is confined to the skin, the following skin-directed therapies are recommended (and often in combination):

• 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 measures, such as the use of interferons, retinoids, etc.
• Biological therapy

Systemic therapy is generally used for higher or advanced stage tumors. A combination of treatment measures may be effectively used to treat the condition. It may include the following:

• Chemotherapy: This approach uses a combination of drugs to kill the cancerous cells and can be used in patients, for all stages of Syringotropic 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 reproductive age could benefit from counseling regarding fertility issues, since some chemotherapy agents have the potential to cause infertility in both genders. There can be permanent damage to the testicles and ovaries, harming their ability to produce sperms or ova. If needed, a patient should discuss sperm or ovum banking options before undergoing chemotherapy. The healthcare provider may help assess the risk-benefit analysis, depending upon each individual’s specific circumstances.

• Radiation therapy: The use of high-energy radiation waves to damage the DNA of cancer cells and kill them
  • This treatment modality is generally used for early stage lymphomas. It is most commonly 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 Syringotropic Mycosis Fungoides, surgery is a very uncommon 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 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 combination with other treatment measures, these can help combat the symptoms of immunodeficiency

If Syringotropic 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: Administering high doses of chemotherapy or radiotherapy may be recommended for systemic cases of Syringotropic MF. However, high doses of chemotherapy drugs have the potential to damage the bone marrow, severely hampering it from making new blood cells. Therefore, before starting high-dose chemotherapy, the healthcare specialist may aspirate some of the patient’s bone marrow (bone marrow harvesting) and freeze/preserve it. After high dose chemotherapy or radiation, the bone marrow is thawed and injected back into patient through a drip (transfusion)
  • Autologous bone marrow transplant - when an individual’s own bone marrow is injected back into him/her after chemotherapy
  • Allogeneic bone marrow transplant - when the condition recurs following transplant using one’s own bone marrow, a donor’s bone marrow (usually from a brother or sister) is used
• Stem cell transplantation: This procedure is similar to bone marrow transplantation and involves the transplantation of healthy blood-forming stem cells into the body. The procedure is also called hematopoietic progenitor cell transplantation. Stem cells can be collected from the bone marrow, circulating (peripheral) blood, and umbilical cord blood. Similar to bone marrow transplantation, stem cells could also originate
  • Either from the patient (autologous)
  • Or a matching donor (allogeneic), if the condition recurs after stem cell transplant using one’s own cells

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 chances of recurrence of the condition may be reduced.

• In order to prevent infections owing to immunosuppression by Syringotropic Mycosis Fungoides or its treatment, it may be recommended that a patient be kept in an isolated ward and treated with appropriate antibiotics
• Nowadays, targeted therapies are being developed that could selectively kill the cancer cells. Many of them are in different stages of clinical trials
• Clinical trials: There may be some newer treatment options, currently on clinical trials, which could 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. Also, follow-up care with regular screening and check-ups are important post-treatment.

How can Syringotropic Mycosis Fungoides be Prevented?

Currently, the cause of Syringotropic Mycosis Fungoides is unknown. As a result, there are no guidelines available for the prevention of the condition. Eliminating some modifiable risk factors may help in preventing the condition, which include:

• Smoking cessation
• Prompt treatment for skin conditions, such as eczema, or viral infections
• Using suitable protective wear (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
• Adhering to a healthy diet
• Regular physical activity, exercise
• Avoidance of unnecessary exposure to chemicals

Keeping a record of the diagnosis, staging, treatment, side effects, etc., is generally recommended, as it helps the patient effectively communicate with the healthcare professional, in case of change of specialist, facility, or recurrence of the condition.

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

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

• The prognosis of Syringotropic Mycosis Fungoides depends upon several factors, which include:
  • Stage of tumor: Lower-stage tumors confined to site of origin, are reported to have a better outcome with prompt and appropriate treatment, than the higher-stage tumors, such as tumors with metastasis
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
  • Age of the individual: Older individuals generally have 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 poorer 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 recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
  • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
  • Progression of the condition makes the outcome worse (progressive Syringotropic Mycosis Fungoides)
• An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment
• The combination chemotherapy drugs used, may have some severe side effects (such as cardio-toxicity). This chiefly impacts the elderly adults, or those who are already affected by other medical conditions. Tolerance to the chemotherapy sessions is a positive influencing factor
• Progression to bone marrow failure is usually associated with short survival

Additional and Relevant Useful Information for Syringotropic Mycosis Fungoides:

• There are many variants of Mycosis Fungoides and they include the following:
  • Pagetoid Reticulosis
  • Syringotropic Mycosis Fungoides
  • Folliculotropic Mycosis Fungoides
  • Granulomatous Mycosis Fungoides
• T-cell lymphomas are less common than B-cell lymphomas
• Treatment for Syringotropic Mycosis Fungoides can cause physical and emotional distress; supportive care and encouragement, help positively and can bring a measure of relief to the patients

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

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