This was about 3-1/2 weeks into Ava's swollen eyelid about when it turned from normal skin tone to red. This is about the time we took her to the pediatrician who referred us to our eye Dr. who eventually referred us back to our pediatrician...and so on.
In a healthy person, these cells help defend against infection. But in histiocytosis, the cells multiply so quickly that they build up and can damage the body instead of protect it.
Except for their rapid growth, the cells are normal. This disease is not cancer. But it is similar. Doctors who treat cancer and blood diseases also treat histiocytosis.
Some people with the disease have excess cells and damage in only one site or organ in their bodies. Others have excess cells in many places throughout their bodies.
The bones are the most common sites of damage - mainly the bones in the skull but also in other places, such as the spine, arms and legs. This disease can affect many body systems.
About 80% to 90% of children recover from this disease with treatment. Sometimes it goes away slowly on its own. It can be serious, even fatal in severe cases. It can also come back later in life.
Langerhans cell histiocytosis has had other names, and there are subtypes of the disease. So you may hear or see one of these names:
* Diffuse reticuloendotheliosis
* Eosinophilic granuloma
* Hand-Schüller-Christian disease
* Hashimoto-Pritzker syndrome
* Histiocytosis X
* Langerhans cell granulomatosis
* Letterer-Siwe disease
* Non-lipid reticuloendotheliosis
* Type II histiocytosis
Langerhans Cell Histiocytosis in Children
A person of any age can get histiocytosis, but it is more common in children. About three-quarters of people with the disease get it before age 10. It is most common in children 1 to 3 years old.
Doctors do not know what causes this disease, but are actively researching the causes and treatment for histiocytosis. It may be an abnormality in the person's immune system but it is not inherited or communicable, passed from person to person.
* Pain, swelling or lump in a bone that does not go away, such as on the skin, arms or legs
* Bone fracture for no clear reason or from only minor trauma
* Loose teeth when you would not expect this, or swollen gums
* Ear infection, cysts in the ear or fluid that oozes from the ear
* Skin rash, such as on the scalp or buttocks
* Swollen lymph nodes in the neck (also called swollen glands)
* Liver problems, which may cause jaundice (yellow color in the whites of the eyes; maybe yellow tint in the skin for some skin colors), fluid in the belly, diarrhea or vomiting
* Bulging eyes or other eye problems
* Cough and trouble breathing
* Failure to thrive, which means not gaining weight and growing normally
* Not wanting to eat or having problems feeding
* Needing to urinate more often than normal and being very thirsty (signs of a health problem called diabetes insipidus)
* Basic signs of illness, such as fever, fatigue and weakness
Langerhans Cell Histiocytosis Diagnosis
It can be hard for doctors to diagnose this disease because it is rare.
Surgery
Doctors may operate to remove the excess Langerhans cells (used mostly for bones).
Steroids
Steroid medication may be injected into the places with excess Langerhans cells.
Anti-Cancer Medicines
Chemotherapy medicines used to kill cancer cells may also help control Langerhans cells. Doctors give some types of these medicines through a vein (intravenously, or by IV). For skin problems, they may apply medicines right on the skin. The doses are lower than used for cancer.
After the 3rd week eyelid got progressively worse and developed a large bump from her eyelid through her brow area and above the brow about 1-1/2" to 2" area. You can see this in the picture below. The picture below is week 6 (July 7th, 2009) and also the same day that we made the decision to go to the emergency room at Fairbanks Memorial Hospital. After a CT scan was done it was recommended that Ava medavac to Children's Hospital in Seattle immediately
Below: Post biopsy by Opthamologist Avery Weiss at Children's Hospital in Seattle
On July 14th, 2009 Ava was diagnosed with Langerhans Cell Histiocytosis. She had a tumor type growth above her eye and it affected her lymph nodes as well, both on her left side. There are also questionable lesions on her scalp.
What is Langerhans Cell Histiocytosis?
NOTE: The information below has been taken from the following websites:
NOTE: The information below has been taken from the following websites:
In Langerhans cell histiocytosis, certain white blood cells, called Langerhans cells, grow out of control.
In a healthy person, these cells help defend against infection. But in histiocytosis, the cells multiply so quickly that they build up and can damage the body instead of protect it.
Except for their rapid growth, the cells are normal. This disease is not cancer. But it is similar. Doctors who treat cancer and blood diseases also treat histiocytosis.
Some people with the disease have excess cells and damage in only one site or organ in their bodies. Others have excess cells in many places throughout their bodies.
The bones are the most common sites of damage - mainly the bones in the skull but also in other places, such as the spine, arms and legs. This disease can affect many body systems.
About 80% to 90% of children recover from this disease with treatment. Sometimes it goes away slowly on its own. It can be serious, even fatal in severe cases. It can also come back later in life.
Langerhans cell histiocytosis has had other names, and there are subtypes of the disease. So you may hear or see one of these names:
* Diffuse reticuloendotheliosis
* Eosinophilic granuloma
* Hand-Schüller-Christian disease
* Hashimoto-Pritzker syndrome
* Histiocytosis X
* Langerhans cell granulomatosis
* Letterer-Siwe disease
* Non-lipid reticuloendotheliosis
* Type II histiocytosis
Langerhans Cell Histiocytosis in Children
A person of any age can get histiocytosis, but it is more common in children. About three-quarters of people with the disease get it before age 10. It is most common in children 1 to 3 years old.
Doctors do not know what causes this disease, but are actively researching the causes and treatment for histiocytosis. It may be an abnormality in the person's immune system but it is not inherited or communicable, passed from person to person.
Symptoms of Langerhans Cell Histiocytosis
Symptoms of Langerhans cell histiocytosis depend on which tissues and organs it affects. Not all children with the disease have the same symptoms.
These are some symptoms that may occur in a child with this disease:
Symptoms of Langerhans cell histiocytosis depend on which tissues and organs it affects. Not all children with the disease have the same symptoms.
These are some symptoms that may occur in a child with this disease:
* Pain, swelling or lump in a bone that does not go away, such as on the skin, arms or legs
* Bone fracture for no clear reason or from only minor trauma
* Loose teeth when you would not expect this, or swollen gums
* Ear infection, cysts in the ear or fluid that oozes from the ear
* Skin rash, such as on the scalp or buttocks
* Swollen lymph nodes in the neck (also called swollen glands)
* Liver problems, which may cause jaundice (yellow color in the whites of the eyes; maybe yellow tint in the skin for some skin colors), fluid in the belly, diarrhea or vomiting
* Bulging eyes or other eye problems
* Cough and trouble breathing
* Weight loss for no reason
* Failure to thrive, which means not gaining weight and growing normally
* Not wanting to eat or having problems feeding
* Needing to urinate more often than normal and being very thirsty (signs of a health problem called diabetes insipidus)
* Basic signs of illness, such as fever, fatigue and weakness
The same symptoms can also be caused by other health problems. So it's important for a child with symptoms like these to see a doctor to find out the cause.
Langerhans Cell Histiocytosis Diagnosis
It can be hard for doctors to diagnose this disease because it is rare.
Your child's doctor will start with a thorough exam to look for signs of illness and will ask about your child's health background. Then the doctor may suggest a number of tests to tell what is happening in your child's body.
Blood tests are a common step. They can give information about what is going on in the blood and the bone marrow, as well as in some organs, such as the liver.
Your child's doctor may also want your child to have pictures taken of the inside of her body, such as an X-ray, CT scan or MRI.
These are called imaging studies, and they allow the doctor to look for areas of disease, such as damage to your child's bones, lungs or brain.
If the doctor thinks that your child may have histiocytosis, the doctor will perform a biopsy to confirm this. This may mean taking out a small piece of bone, lung or skin or a lymph node.
Treatment for Langerhans cell histiocytosis
Treatment for Langerhans cell histiocytosis depends on how many parts of the body are affected and which parts. Here are the main treatments that doctors use. All of these are offered through Children's Hospital.
Treatment Options for Langerhans Cell Histiocytosis
Surgery
Doctors may operate to remove the excess Langerhans cells (used mostly for bones).
Steroids
Steroid medication may be injected into the places with excess Langerhans cells.
Anti-Cancer Medicines
Chemotherapy medicines used to kill cancer cells may also help control Langerhans cells. Doctors give some types of these medicines through a vein (intravenously, or by IV). For skin problems, they may apply medicines right on the skin. The doses are lower than used for cancer.
This treatment can help control cells that are damaging bone and making it weak. The doses are lower than for cancer.
New Treatments for Langerhans Cell Histiocytosis
Children's Hospital is working with the Histiocyte Society to develop better treatments for Langerhans.
Children's Hospital is working with the Histiocyte Society to develop better treatments for Langerhans.
