Frequently Asked Questions


LCH in Adults

The questions below are regarding LCH in Adults specifically. Please click on a question to view the answer.

  1. What causes LCH?
  2. Is there a cure for LCH?
  3. What is considered to be remission?
  4. Is LCH fatal?
  5. Does LCH spread?
  6. Is LCH hereditary?
  7. Where did LCH get its name?
  8. What are the different therapies/treatments commonly used to treat LCH?
  9. Is there a blood test to diagnose LCH?
  10. With the diagnosis and treatment of LCH, am I more likely to develop cancer?
  11. With a history of LCH, can I become pregnant?
  12. What are permanent consequences of LCH?
  13. What causes chronic pain in adults with LCH?
  14. What healthcare workers should I see to help with pain management?
  15. What should I look for in a doctor?
  16. Is it true that LCH is mostly a childhood disease?
  17. What is “PLCH?”
  18. Why is cigarette smoking dangerous for me?
  19. Can adults with a history of LCH donate blood or bone marrow?
  20. What is neurodegeneration?
  21. Can neurodegeneration be prevented/reversed/treated?
  22. What is chemo brain?

Possible Side Effects of Treatment

  1. What are the possible side effects of vinblastine?
  2. What are the possible side effects of prednisone?
  3. What are the possible side effects of methotrexate?
  4. What are the possible side effects of 6-MP (mercaptopurine)?
  5. What are the possible side effects of 2-CdA (cladribine/leustatin)?

 

Histiocytic Disorders and Orphan Diseases

The questions below are regarding the Histiocytic Disorders in general, as well as Orphan Diseases. Please click on the question to view the answer.

  1. What are histiocytic disorders, and how are they classified?
  2. Why are all of these diseases with different names considered to be related to each other?
  3. Where can I find reliable information about histiocytosis?
  4. How can I explain histiocytosis to family and friends?
  5. What is an orphan disease?
  6. How many orphan diseases are there?
  7. Where can I learn more about rare diseases in general?



LCH in Adults

  1. What causes LCH?
    To date, the cause has not been determined. Multiple potential causes have been explored, including viruses, molds, infections, genetics, geographic location, racial clustering, seasonal changes, and environmental exposure. Results of these studies are still insufficient to provide a definitive answer. Further studies are needed.

  2. Is there a cure for LCH?
    While some patients go into remission and may live normal lives with or without treatment, we usually don’t use the term “cure” with this disease. No specific amount of time without active disease has yet been established for adults to determine when a patient is considered to be cured.

  3. What is considered to be remission?
    Complete remission means that there is no evidence of disease, whereas partial remission means that most of the signs and symptoms of LCH are gone, but some still remain. Doctors use the term response and “non-active” to describe patients who are free of symptoms and signs of LCH. Usually a cure is linked to being in remission for a certain period of time. There is no established period of “non-active” disease before LCH is considered cured, but the chance for recurrence is low after five years from end of treatment.

  4. Is LCH fatal?
    It can be. A small percentage of patients, most often those with multisystem risk-organ involvement that is unresponsive to treatment, may not survive.

  5. Does LCH spread?
    The exact mechanism that causes lesions to appear in other locations in the body is not yet known. However, some researchers believe that abnormal LCH cells travel through the blood like tumor cells and “seed” in different locations, creating new lesions. Others believe that there are different manifestations of the same disease.

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  6. Is LCH hereditary?
    Although there are rare families (less than 2% of all cases) documented with more than one member diagnosed with LCH, at this point, there is no clear evidence that this disease is inherited.

  7. Where did LCH get its name?
    In 1868, the German pathologist Paul Langerhans discovered a type of white blood cell which eventually came to bear his name. The various manifestations of LCH were previously known by a number of different names (histiocytosis-X, eosinophilic granuloma, Letterer-Siwe disease, Hand-Schüller-Christian syndrome, etc.). In 1983, it was suggested that this disorder be named “Langerhans cell histiocytosis,” to recognize the key role of the Langerhans cell in all of the different manifestations. This name was later approved by the scientists comprising the Histiocyte Society.

  8. What are the different therapies/treatments commonly used to treat LCH?
    Treatment is based upon the organ(s) involved, extension of disease, and in some cases, age of the patient. In some cases, no treatment is necessary. Others may respond to surgical removal, steroids, or anti-inflammatory drugs (NSAIDs). Low-dose radiation is helpful in some situations, but should be carefully used in children. There are patients who require chemotherapy such as vinblastine, vincristine, etoposide (VP-16), methotrexate, cytosine-arabinoside (Ara-C), and/or 6-MP. In patients with severe disease that does not respond to initial treatment, stronger chemotherapy combinations may be used. Ultraviolet light (PUVA) may be helpful for limited skin disease. In very rare instances, a transplant of the liver, lung, or bone marrow may be necessary.

  9. Is there a blood test to diagnose LCH?
    LCH is diagnosed with a biopsy of the affected tissue. Blood tests may be done to help determine the extent and/or severity of involvement, but blood tests are not diagnostic of the disease.

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  10. With the diagnosis and treatment of LCH, am I more likely to develop cancer?
    Although this occurs rarely, LCH is associated with cancer more often than would be expected by chance. This can occur before, during, or after the diagnosis of LCH. Some cancers following the LCH diagnosis might be related to the treatments given. When cancer occurs before LCH, the histiocytosis might represent a “reaction” to the cancer itself.

  11. With a history of LCH, can I become pregnant?
    Drugs used as part of the usual LCH treatment are not associated with infertility. These include vinblastine, vincristine, 2-CdA, methotrexate, Ara-C, and etoposide (VP-16). LCH can, however, affect the endocrine system, and complications can result in infertility. This is especially true if hormone loss is not diagnosed in time and if no hormone replacement is given. However, in some cases, hormone replacement therapy may restore fertility.

  12. What are permanent consequences of LCH?
    Permanent consequences are also known as late effects of LCH, although they can occur early on. They are believed to be mostly related to the disease rather than treatment and include:

    • Diabetes Insipidus
    • Stunted growth
    • Bone abnormalities
    • Hearing loss
    • Neurological problems, including poor coordination, unsteadiness, difficulty with handwriting, abnormal eye movements, problems with speech, learning disabilities/decreased school performance, memory loss, and behavior difficulties
    • Loss of teeth
    • Loss of spinal height
    • Delayed puberty
    • Bulging eyes
    • Scarring of lungs
    • Scarring of liver/cirrhosis
    • Secondary cancers
    Read more about permanent consequences of LCH

  13. What causes chronic pain in adults with LCH?
    Some pain and cramping can be a side effect of treatment, such as vinblastine and steroids. Pain may also be directly related to active disease. In cases of more chronic pain, some researchers suspect that cytokines, which are a type of messenger, stimulate white blood cells to release inflammatory molecules that produce pain.

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  14. What healthcare workers should I see to help with pain management?
    The primary oncologist/physician can treat and manage chronic pain in many cases. However, if the pain does not respond to the usual therapy and/or if the pain compromises quality of life, referral to a pain clinic may be necessary. Clinics differ in their approach and what they offer, but most include a team of health care providers with a variety of ways to manage pain and restore quality of life.

  15. What should I look for in a doctor?
    Many adults with LCH have been seen by a number of specialists, depending on their symptoms. It will be important to find a physician knowledgeable about adult LCH who is willing to serve as the primary “hub” and coordinate input from the specialists. Ideally, this physician would be an oncologist who specializes in cancer-like illnesses and is qualified to provide systemic treatment. Accessibility and good communication skills with you, as well as other physicians, are important. Find a Physician in your area today.

  16. Is it true that LCH is mostly a childhood disease?
    Not necessarily. Although we do know the incidence of childhood LCH, there is not enough data to determine how many adults are affected by this disease.

  17. What is “PLCH?”
    PLCH (Pulmonary Langerhans cell histiocytosis) is LCH of the lung. It affects mostly adults who smoke and often occurs without other LCH involvement.

  18. Why is cigarette smoking dangerous for me?
    It is believed that there is a strong link between cigarette smoking and pulmonary LCH. An estimated 90-95% of pulmonary-only LCH patients currently smoke or have smoked. Although the exact mechanism is not understood, it is believed that smoking can cause an accumulation of Langerhans cells in the lungs.

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  19. Can adults with a history of LCH donate blood or bone marrow?
    Although there is no data to prove a risk, adults with a history of LCH are advised against donating blood or platelets. They should also not be organ donors.

  20. What is neurodegeneration?
    Neurodegeneration is progressive loss of brain function. It occurs as a permanent consequence in some cases of LCH.

  21. Can neurodegeneration be prevented/reversed/treated?
    It is not currently known whether neurodegeneration can be prevented. It is believed that neurodegeneration cannot be reversed, and there is controversy whether patients with neurodegeneration can be successfully treated. There have been some promising results with Ara-C but more extensive scientific studies are required.

  22. What is chemo brain?
    Chemo brain is a term used for mild brain impairment that may occur during treatment. It can include symptoms such as difficulty concentrating, feeling disorganized, trouble with multi-tasking, memory lapses, slower thinking, word-finding difficulty, etc. For most patients, it lasts a short time.

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Possible Side Effects of Treatment

  1. What are the side effects of vinblastine?
    Side effects include:
    • Low blood counts (with higher risk of infection)
    • Mild nausea/vomiting/constipation
    • Easily sunburned.Skin irritation at site of injection
    • Thin or brittle hair
    • Fatigue
    • Bone pain
    • Hoarseness
    • Seizures
    • Shortness of breath
    • Nerve damage (especially in adults) with tingling, numbness and/or pain of the hands and feet
  2. What are the side effects of prednisone?
    Side effects include:
    • Increase in blood sugar
    • Increase in appetite
    • Heartburn
    • Bloating/fluid retention/weight gain
    • Difficulty sleeping
    • Mood/behavior/personality changes
    • Higher risk of infection
    • Slow wound healing
    • Muscle weakness
    • Loss of bone calcium
    • Increased hair growth
    More unusual side effects may include:
    • Problems with vision/eye pain
    • Seizures
    • Confusion
    • Muscle twitching

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  3. What are the side effects of methotrexate?
    • Side effects include:
    • Mouth sores/swollen, tender gums
    • Nausea/vomiting/diarrhea/decreased appetite
    • Low blood counts
    • Dizziness/drowsiness
    • Headache
    More unusual side effects may include:
    • Blurred vision or loss of vision
    • Seizures
    • Confusion
    • Weakness/difficulty moving one or both sides of the body
    • Loss of consciousness
    • Lung damage
    • Allergic reactions
  4. What are the possible side effects of 6-MP (mercaptopurine)?
    More common signs/symptoms include:
    • Low blood counts (red cells, white cells, and clotting cells)
    • Nausea/vomiting/decreased appetite
    • Headache Weakness/fatigue/achiness
    • Rash/darkening of the skin
  5. What are the possible side effects of 2-CdA (cladribine/leustatin)?
    More common signs/symptoms include:
    • Flu-like symptoms (Fever, chills, headache, fatigue, nausea/vomiting)
    • Decreased appetite
    • Constipation
    • Low blood counts (red cells, white cells, and clotting cells)
    • Skin rash/redness/itching

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Histiocytic Disorders and Orphan Diseases

  1. What are histiocytic disorders, and how are they classified?
    Histiocytic disorders are a diverse group of diseases caused by over-production of white blood cells known as histiocytes, which can lead to organ damage and tumor formation. They include a wide variety of conditions that can affect both children and adults.

    The disorders are classified into three groups based on the types of histiocyte cells involved.
    • The first group is called a dendritic cell disorder, and the most common disease in this group is Langerhans cell histiocytosis. Also included in this group are more rare diseases, juvenile xanthogranuloma (JXG) and Erdheim Chester.
    • The second group is called a macrophage cell disorder, and includes primarily hemophagocytic lymphohistiocytosis (HLH) and Rosai-Dorfman.
    • The third group is called malignant histiocytosis and includes certain kinds of leukemia and tumors.
  2. Why are all of these diseases with different names considered to be related to each other?
    All of the diseases are caused by the over-production of white blood cells called histiocytes. Their different classifications depend on the type of histiocyte involved.

  3. Where can I find reliable information about histiocytosis?
    The Histiocytosis Association’s online community provides a number of informational documents and articles, as well as links to medical articles about the histiocytic disorders. While the Internet does provide a significant volume of information about histiocytic disorders, some of this information is not accurate. It is important to look for documents that are current, are free of grammatical and spelling errors, appear to be objective, are free of advertisements, and clearly state their sources.

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  4. How can I explain histiocytosis to family and friends?
    Histiocytosis is a rare disease that is caused by the over-production of a type of white cell that can lead to organ damage and the formation of tumors. The Histiocytosis Association’s Disease Fact Sheets are also a great way to help explain these complicated diseases to family and friends.

  5. What is an orphan disease?
    According to the Rare Disease Act of 2002, an orphan disease, also known as a rare disease, affects less than 200,000 persons in the U.S., or less than 1 in 1500 people. The criteria may vary in other countries. For example in Europe, an orphan disease is defined to occur in less than 1 in 2000.

  6. How many orphan diseases are there?
    According to the National Institutes of Health there are approximately 6800 such diseases. Combined, they affect nearly 30 million Americans.

  7. Where can I learn more about rare diseases in general?

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