Frequently Asked Questions

 

Rosai-Dorfman Disease

The questions below are regarding Rosai-Dorfman Disease specifically. Please click on a question to view the answer.

  1. What causes Rosai-Dorfman?
  2. Is there a cure for Rosai-Dorfman?
  3. What are the different therapies/treatments commonly used to treat Rosai-Dorfman?
  4. Can an infant be tested at birth for Rosai-Dorfman?
  5. What causes chronic pain in adults with Rosai-Dorfman?

Possible Side Effects of Treatment

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

Histiocytic Disorders and Orphan Diseases

The questions below are regarding Histiocytic Disorders and Orphan Diseases in general. Please click on a 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?

Rosai-Dorfman Disease

 

  1. What causes Rosai-Dorfman?
    Rosai-Dorfman involves over-production of a type of white blood cell called a non-Langerhans sinus histiocyte. The cause of this over-production is not yet known.

  2. Is there a cure for Rosai-Dorfman?
    While many patients go into remission and live normal lives with or without treatment, we usually don’t use the term “cure.” There is no established period of inactive disease before RDD is considered cured.

  3. What are the different therapies/treatments commonly used to treat Rosai-Dorfman?
    Many Rosai-Dorfman patients do not require treatment. Some patients with severe or persistent disease may need treatment with surgery, steroids, and/or chemotherapy. Rarely, radiation therapy is used.

  4. Can an infant be tested at birth for Rosai-Dorfman?
    A biopsy of the affected tissue, rather than a blood test, is required for diagnosis and would therefore not be appropriate as a routine test unless this disease is suspected.

  5. What causes chronic pain in adults with Rosai-Dorfman?
    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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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

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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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