lunes, 26 de septiembre de 2016

Langerhans Cell Histiocytosis Treatment (PDQ®)—Patient Version - National Cancer Institute

Langerhans Cell Histiocytosis Treatment (PDQ®)—Patient Version - National Cancer Institute





National Cancer Institute

Langerhans Cell Histiocytosis Treatment (PDQ®)–Patient Version





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General Information About Langerhans Cell Histiocytosis (LCH)

KEY POINTS

  • Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body.
  • Family history or having a parent who was exposed to certain chemicals may increase the risk of LCH.
  • The signs and symptoms of LCH depend on where it is in the body.
    • Skin and nails
    • Mouth
    • Bone
    • Lymph nodes and thymus
    • Endocrine system
    • Central nervous system (CNS)
    • Liver and spleen
    • Lung
    • Bone marrow
  • Tests that examine the organs and body systems where LCH may occur are used to detect (find) and diagnose LCH.
  • Certain factors affect prognosis (chance of recovery) and treatment options.


Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body.

Langerhans cell histiocytosis (LCH) is a rare cancer that begins in LCH cells (a type ofdendritic cell which fights infection). Sometimes there are mutations (changes) in LCH cells as they form. These include mutations of the BRAF gene. These changes may make the LCH cells grow and multiply quickly. This causes LCH cells to build up in certain parts of the body, where they can damage tissue or form lesions.
LCH is not a disease of the Langerhans cells that normally occur in the skin.
LCH may occur at any age, but is most common in young children. Treatment of LCH in children is different from treatment of LCH in adults. The treatments for LCH in childrenand adults are described in separate sections of this summary.
Check the list of NCI-supported cancer clinical trials that are now accepting patients withchildhood Langerhans cell histiocytosis. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.

Family history or having a parent who was exposed to certain chemicals may increase the risk of LCH.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for LCH include the following:
  • Having a parent who was exposed to certain chemicals such as benzene.
  • Having a parent who was exposed to metal, granite, or wood dust in the workplace.
  • family history of cancer, including LCH.
  • Having infections as a newborn.
  • Having a personal history or family history of thyroid disease.
  • Smoking, especially in young adults.
  • Being Hispanic.

The signs and symptoms of LCH depend on where it is in the body.

These and other signs and symptoms may be caused by LCH or by other conditions. Check with your doctor if you or your child have any of the following:

Skin and nails

LCH in infants may affect the skin only. In some cases, skin-only LCH may get worse over weeks or months and become a form called high-risk multisystem LCH.
In infants, signs or symptoms of LCH that affects the skin may include:
  • Flaking of the scalp that may look like “cradle cap”.
  • Raised, brown or purple skin rash anywhere on the body.
In children and adults, signs or symptoms of LCH that affects the skin and nails may include:
  • Flaking of the scalp that may look like dandruff.
  • Raised, red or brown, crusted rash in the groin area, abdomen, back, or chest, that may be itchy.
  • Bumps or ulcers on the scalp.
  • Ulcers behind the ears, under the breasts, or in the groin area.
  • Fingernails that fall off or have discolored grooves that run the length of the nail.

Mouth

Signs or symptoms of LCH that affects the mouth may include:
  • Swollen gums.
  • Sores on the roof of the mouth, inside the cheeks, or on the tongue or lips.
  • Teeth that become uneven.
  • Tooth loss.

Bone

Signs or symptoms of LCH that affects the bone may include:
  • Swelling or a lump over a bone, such as the skull, ribs, spine, thigh bone, upper arm bone, elbow, eye socket, or bones around the ear.
  • Pain where there is swelling or a lump over a bone.
Children with LCH lesions in bones around the ears or eyes have a high risk for diabetes insipidus and other central nervous system disease.

Lymph nodes and thymus

Signs or symptoms of LCH that affects the lymph nodes or thymus may include:
  • Swollen lymph nodes.
  • Trouble breathing.
  • Superior vena cava syndrome. This can cause coughing, trouble breathing, and swelling of the face, neck, and upper arms.

Endocrine system

Signs or symptoms of LCH that affects the pituitary gland may include:
  • Diabetes insipidus. This can cause a strong thirst and frequent urination.
  • Slow growth.
  • Early or late puberty.
  • Being very overweight.
Signs or symptoms of LCH that affects the thyroid may include:
  • Swollen thyroid gland.
  • Hypothyroidism. This can cause tiredness, lack of energy, being sensitive to cold,constipation, dry skin, thinning hair, memory problems, trouble concentrating, anddepression. In infants, this can also cause a loss of appetite and choking on food. In children and adolescents, this can also cause behavior problems, weight gain, slow growth, and late puberty.
  • Trouble breathing.

Central nervous system (CNS)

Signs or symptoms of LCH that affects the CNS (brain and spinal cord) may include:
  • Loss of balance, uncoordinated body movements, and trouble walking.
  • Trouble speaking.
  • Trouble seeing.
  • Headaches.
  • Changes in behavior or personality.
  • Memory problems.
These signs and symptoms may be caused by lesions in the CNS or by CNSneurodegenerative syndrome.

Liver and spleen

Signs or symptoms of LCH that affects the liver or spleen may include:
  • Swelling in the abdomen caused by a buildup of extra fluid.
  • Trouble breathing.
  • Yellowing of the skin and whites of the eyes.
  • Itching.
  • Easy bruising or bleeding.
  • Feeling very tired.

Lung

Signs or symptoms of LCH that affects the lung may include:
  • Collapsed lung. This condition can cause chest pain or tightness, trouble breathing, feeling tired, and a bluish color to the skin.
  • Trouble breathing, especially in adults who smoke.
  • Dry cough.
  • Chest pain.

Bone marrow

Signs or symptoms of LCH that affects the bone marrow may include:
  • Easy bruising or bleeding.
  • Fever.
  • Frequent infections.

Tests that examine the organs and body systems where LCH may occur are used to detect (find) and diagnose LCH.

The following tests and procedures may be used to detect (find) and diagnose LCH or conditions caused by LCH:
  • Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
  • Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person's mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
  • Complete blood count (CBC) with differential : A procedure in which a sample ofblood is drawn and checked for the following:
  • Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the body by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
  • Liver function test : A blood test to measure the blood levels of certain substances released by the liver. A high or low level of these substances can be a sign of disease in the liver.
  • BRAF gene testing : A laboratory test in which a sample of blood or tissue is tested for mutations of the BRAF gene.
  • Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, red blood cells, and white blood cells.
  • Water deprivation test : A test to check how much urine is made and whether it becomes concentrated when little or no water is given. This test is used to diagnose diabetes insipidus, which may be caused by LCH.
  • Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone. A pathologist views the bone marrow and bone under a microscope to look for signs of LCH.
    ENLARGEBone marrow aspiration and biopsy; drawing shows a patient lying face down on a table and a bone marrow needle being inserted into the hip bone. Inset shows the bone marrow needle being inserted through the skin into the bone marrow of the hip bone.
    Bone marrow aspiration and biopsy. After a small area of skin is numbed, a bone marrow needle is inserted into the patient’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.
    The following tests may be done on the tissue that was removed:
    • Immunohistochemistry : A test that uses antibodies to check for certain antigensin a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.
    • Flow cytometry : A laboratory test that measures the number of cells in a sample, how many cells are live, and the size of the cells. It also shows the shapes of the cells and whether there are tumor markers on the surface of the cells. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before alaser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.
  • Bone scan : A procedure to check if there are rapidly dividing cells in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.
    ENLARGEBone scan; drawing shows a child lying on a table that slides under the scanner, a technician operating the scanner, and a computer monitor that will show images made during the scan.
    Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.
  • X-ray : An x-ray of the organs and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. Sometimes a skeletal survey is done. This is a procedure to x-ray all of the bones in the body.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
    ENLARGEComputed tomography (CT) scan of the abdomen; drawing shows the patient on a table that slides through the CT machine, which takes x-ray pictures of the inside of the body.
    Computed tomography (CT) scan of the abdomen. The patient lies on a table that slides through the CT machine, which takes x-ray pictures of the inside of the body.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A substance called gadolinium may be injected into a vein. The gadolinium collects around the LCH cells so that they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
    ENLARGEMagnetic resonance imaging (MRI) of the abdomen; drawing shows a child lying on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.
    Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.
  • PET scan (positron emission tomography scan): A procedure to find tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
    ENLARGEPositron emission tomography (PET) scan; drawing shows a child lying on table that slides through the PET scanner.
    Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child's vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
  • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
    ENLARGEAbdominal ultrasound; drawing shows a woman on an exam table during an abdominal ultrasound procedure. A diagnostic sonographer (a person trained to perform ultrasound procedures) is shown passing a transducer (a device that makes sound waves that bounce off tissues inside the body) over the surface of the patient’s abdomen. A computer screen shows a sonogram (computer picture).
    Abdominal ultrasound. An ultrasound transducer connected to a computer is passed over the surface of the abdomen. The ultrasound transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).
  • Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and alens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
  • Endoscopy : A procedure to look at organs and tissues inside the body to check forabnormal areas in the gastrointestinal tract or lungs. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease.
  • Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for LCH cells. To diagnose LCH, a biopsy of bone lesions, skin, lymph nodes, or the liver may be done.

Certain factors affect prognosis (chance of recovery) and treatment options.

LCH in organs such as the skin, bones, lymph nodes, or pituitary gland usually gets better with treatment and is called "low- risk". LCH in the spleen, liver, or bone marrow is harder to treat and is called "high-risk".
The prognosis (chance of recovery) and treatment options depend on the following:
  • How old the patient is when diagnosed with LCH.
  • How many organs or body systems the cancer affects.
  • Whether the cancer is found in the liver, spleen, bone marrow, or certain bones in the skull.
  • How quickly the cancer responds to initial treatment.
  • Whether the cancer has just been diagnosed or has come back (recurred).
In infants up to one year of age, LCH may go away without treatment.


  • Updated: April 6, 2016

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