Has your little one developed a small lump somewhere on their body? Or a skin rash or itchy rash that won't heal? Sometimes these are normal, but rarely they can be signs of a rare condition called Langerhans Cell Histiocytosis (LCH). It's normal to feel scared when you hear this name, because it's not something we're used to hearing. But don't worry, let's talk about it in simple terms.
Simply put, what is LCH?
Think of our body as a big country. There is an army to protect this country, and that is our immune system . A special type of soldier in this army is called Langerhans cells . These cells are a type of white blood cell. Their main job is to fight germs and infections that enter our body and protect us from disease. These soldiers are found throughout our body, especially in the skin, lungs, lymph nodes, bone marrow, spleen, and liver.
But in the case of LCH, these Langerhans cells multiply uncontrollably and accumulate in different parts of the body. It's like a herd of soldiers just standing around in one place without a fight. When they accumulate like this, they start to damage the healthy tissues in those places. Lesions form in those places.
The good news about this condition is that most children can recover completely from the disease with proper treatment. Sometimes, especially if it only affects the skin, it may go away on its own without any treatment. However, if these cells affect vital organs such as the bone marrow, spleen or liver, more intensive treatment may be required.
Is LCH cancer?
This is a question that many people have. In fact, there is still a bit of disagreement among doctors about this. Many researchers consider LCH to be a condition similar to cancer, that is, a neoplasm . That is, an abnormal growth of cells. But others think that it is an inflammatory disease of the immune system.
However, LCH is treated by oncologists, who specialize in cancer and blood diseases. Sometimes, anti-cancer treatments such as chemotherapy are also used to treat it.
Who is most likely to get this disease?
LCH is most often seen in newborns and children between the ages of 1 and 15. Adults are very rare, but it is not impossible.
Statistically, only one or two out of every million newborns are born with this disease each year. This means that it is a very rare condition.
What are the symptoms of LCH?
LCH does not affect every child in the same way. While some children may only have one area of their body affected, others may have multiple areas of their body affected. Therefore, the symptoms vary depending on the part of the body affected. Let's take a look at what they are.
| Affected body part | Symptoms that can be seen |
|---|---|
| Bones | LCH affects the bones in about 80% of patients. A lump or swelling may appear in places such as the skull, bones around the eyes, behind the ears, jawbone, limbs, spine, and hips. Pain may or may not be present. In addition, headaches, neck/back pain, difficulty walking, and a limp may be seen. |
| Skin | The most common rash is a skin rash. Cradle cap can be a non-healing condition that appears on the baby's head. Red, scaly patches can appear on the groin, armpits, stomach, chest, and back. These may ooze and be itchy or painful. The nails may also change color or fall off. |
| Mouth | Loose or loose teeth, receding teeth, swollen gums, sores on the lips, tongue, inside the cheeks, or on the roof of the mouth. |
| Liver or Spleen | An enlarged liver or spleen can cause abdominal swelling, yellowing of the eyes and skin (jaundice), itching, extreme fatigue, and easy bruising or bleeding. |
| Bone Marrow | Anemia, paleness, fatigue, and loss of appetite due to a decrease in red blood cells. Frequent infections and fever due to a decrease in white blood cells. Easy bruising due to a decrease in platelets. |
| Endocrine System (Endocrine System - Hormones) | If the pituitary gland is affected: excessive thirst and frequent urination (diabetes insipidus), stunted growth, early or delayed puberty, weight gain. If the thyroid gland is affected: swelling of the gland, difficulty breathing. |
| Ears | Frequent ear infections, discharge from the ear, redness of the ear, itching in the ear, ear pain, and hearing loss. |
| Eyes | Bulging eyes, swelling above the eyes, vision impairment. |
| Lymph Nodes | Swelling and pain in lumps (lumps) in the neck, armpits, or groin. |
| Central Nervous System | Headache, dizziness, vomiting, difficulty walking, loss of balance (ataxia), difficulty speaking or seeing, seizures, changes in behavior and memory. |
| Lungs | This is common among adults, especially smokers. Chest pain, dry cough, difficulty breathing, lung collapse (pneumothorax). |
| Gastrointestinal Tract | Stomach pain, vomiting, diarrhea, blood in the stool, and poor growth due to nutritional deficiencies. |
The important thing is that these symptoms can also be seen in many other common diseases. So don't be afraid of LCH just because you have one or two of these symptoms. But if these symptoms persist, it is very important to see a doctor and get a proper diagnosis.
What is the specific cause of LCH?
Simply put, our cells have genes that tell them to "divide now, stop now." About half of children with LCH have a small change, or mutation, in their gene called `BRAF .' This gene makes a protein that controls cell growth. Because of this mutation, that protein doesn't get the command to "stop dividing." It's like the 'on' switch is stuck. So the Langerhans cells keep dividing and merging.
This is not something that is inherited from parents to children. This genetic mutation occurs randomly after the child is conceived in the mother's womb.
In addition to the `BRAF` gene, researchers have found that this disease can also be caused by mutations in other genes such as `MAP2K1`, `RAS`, and `ARAF`.
How do you find this, Doctor?
When you take your child to the doctor, he or she will first ask you about your child's symptoms. Then, they will examine your child carefully. Because LCH can affect many different parts of the body, several tests may be needed to confirm the diagnosis.
| Test type | Simply put... |
|---|---|
| Blood Tests | A complete blood count (CBC) measures the number of red cells, white cells, and platelets in the blood. Blood tests are also done to check things like liver function. |
| Biopsy | This is the most definitive way to confirm LCH. A small piece of tissue is taken from a lump or lesion under anesthesia and examined under a microscope to see if LCH cells are present. If bone marrow involvement is suspected, a bone marrow biopsy may be done. |
| Genetic Testing | The tissue sample taken from the biopsy is used to test for a mutation in the `BRAF` gene. |
| Imaging Tests | Tests such as X-rays, CT scans, MRI scans, and PET scans can determine the extent of LCH's effects on internal organs such as the bones, brain, and lungs. |
Based on the results of these tests, your doctor will refer your child to a pediatric hematologist/oncologist.
What are the treatments for LCH?
Not all children with LCH require the same type of treatment. Treatment depends on the location and severity of the disease.
Doctors classify LCH into two main types:
1. Single-system LCH: The disease affects only one organ system in the body (e.g., only the bones or only the skin).
2. Multi-system LCH: The disease affects two or more organ systems of the body (e.g., skin and liver).
Also, organs such as the liver, spleen, and bone marrow are considered "high-risk" organs, while organs such as the skin, bones, and lungs are considered "low-risk." This classification determines the treatment plan.
There are several main treatment methods:
- Steroid therapy: Especially when only the skin is affected, steroid medications such as prednisone are used as pills or ointments.
- Surgery: Surgery such as curettage is performed to remove an LCH tumor in a bone.
- Chemotherapy: This is a type of medicine that is given to kill cancer cells. Because LCH cells divide rapidly, these medicines stop them from growing. These medicines can be given as pills, injections, or as a cream applied to the skin.
- Radiation therapy: Uses high-energy rays to destroy LCH cells. This is now used very rarely.
- Targeted therapy: For children with the `BRAF` gene mutation, there are drugs (BRAF inhibitors) that target that mutation. These have very little damage to healthy cells.
- Immunotherapy: Stimulating the child's own immune system to fight LCH cells.
- Stem cell transplant: A treatment option considered in very severe cases that are refractory to other treatments.
How is the situation after treatment?
The prognosis for LCH depends on several factors, including which parts of the body the disease has affected, how far it has spread, and how well it responds to treatment.
- The cure rate for children with only "low-risk" organ involvement (both single-system and multi-system) is nearly 100% . This means that there is no risk of death. However, there is a risk of recurrence or other long-term complications.
- The condition of children whose "high-risk" organs, such as the liver, spleen, and bone marrow, are affected may be more serious.
Therefore, it is extremely important to follow-up with your doctor for many years, even after treatment is complete. You need to be regularly checked for recurrence.
Take-Home Message
- Langerhans Cell Histiocytosis (LCH) is a rare disease caused by the uncontrolled growth of a type of immune cell. It is most commonly seen in children.
- Although this is not classified as cancer, oncologists treat it using anti-cancer therapies.
- Symptoms (skin lesions, bone nodules, frequent infections) vary greatly depending on the part of the body affected by the disease.
- A biopsy is essential to definitively diagnose the disease.
- For many children, especially those in the "low-risk" category, treatment can completely cure the disease.
- Even after treatment is complete, it is very important to have medical follow-up for several years to see if the disease recurs.
- Discuss any questions or concerns you have about your child's condition openly with your doctor.
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