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Langerhans cell histiocytosis: symptoms, causes and treatment


Langerhans cell histiocytosis, or histiocytosis X, is a rare condition that occurs primarily in children. It is distinguished by a type of abnormal proliferation of immune cells called histiocytes. This condition mainly affects infants and toddlers. Rarely, it can also occur in adults.

This blog discusses Langerhans cell histiocytosis, its symptoms, causes, and various treatment options.

What is Langerhans cell histiocytosis?

As previously mentioned, Langerhans cell histiocytosis is a rare disease that primarily affects children. Children affected by this disease have an abnormal increase in a specific form of white blood cells known as histiocytes. These cells are an integral part of your child’s immune system, which helps fight a variety of foreign substances and infections. Overproduction of histiocytes can lead to the formation of lesions, tumors, and other tissue damage. there is. The disorder affects several body parts, including bones, and can spread to other areas.

Previously, experts considered Langerhans cell histiocytosis to be a cancer-like condition. However, only recently have doctors recognized it as an autoimmune disease. Children from 1 year old to her 15 years old are affected by this disorder.

What are the symptoms of Langerhans cell histiocytosis?

Signs of Langerhans cell histiocytosis can be found in different parts of the body. But it stands out on skin and bones. It also affects multiple internal organs, including the liver, lungs, brain, spleen, and lymph nodes.

Your child may experience mild to severe symptoms. However, some children develop severe disorders that affect multiple parts of the body.

Here are the signs and symptoms of Langerhans cell histiocytosis.

  1. skin: Children born with this disorder have red, scaly scalps and are often confused with cradle cap. This is a common skin condition. You may see red, scaly bumps in your child’s skin folds.
  2. liver: Severe cases of the disorder affect only the liver. This may cause your child to appear jaundiced or slow blood clots in your child.
  3. Lymph nodes: These glands are located behind the ears, neck, and other parts of the body. With this disorder, your child may experience inflammation of the lymph nodes. In some cases, they may also develop breathing problems and coughing.
  4. Bone: Your child may develop bone lesions, extreme pain, and limp legs.
  5. Teeth and Gums: swollen gums; Your child can also lose teeth.
  6. eye: May affect vision or cause puffy eyes in children
  7. central nervous system: You may find your child complaining constantly and chronically headache,dizzy, vomitingexcessive thirst, frequent urination
  8. ear: Frequent and regular infections and discharges from your child’s ears
  9. Pituitary gland: It resides in the base of the brain where hormones are produced. Hormones are involved in several bodily functions. If the pituitary gland is affected, the child may experience delayed puberty. infertilitypolyuria, and thyroid problem.

Other common signs include:

  1. heat
  2. tired
  3. Malaise
  4. Hypersensitivity
  5. can’t gain weight
  6. anemia
  7. seizure

Other underlying health conditions can also cause the above symptoms. Therefore, it is important to see the right doctor and get a proper diagnosis.


What causes Langerhans cell histiocytosis?

Experts continue to research to find the cause of the disorder. However, nearly half of patients with Langerhans cell histiocytosis carry the rapidly proliferating mutated gene. Genetic mutations occur after birth and are not inherited from parents.

Experts believe other factors play an important role in the development of the disorder, including:

  1. smoking
  2. Parents exposed to environmental toxins such as benzene or wood flour
  3. neonatal infections
  4. History of thyroid disease.

How do doctors diagnose Langerhans cell histiocytosis?

Apart from the physical examination and understanding symptoms, your doctor may also prescribe certain tests.

  1. Imaging test: The pediatrician will take x-rays and CT scan, to check for tumors in some body parts. These tumors have a punched out appearance.
  2. bone marrow biopsy: Your doctor may surgically remove a small sample of your child’s bone marrow to check for certain proteins and other markers of Langerhans cell histiocytosis.
  3. Blood test: It helps determine the health of internal organs such as the kidneys, liver, and thyroid. It also helps determine if the cells in your body are growing rapidly.
  4. Urinalysis: Pathologists look for abnormal levels of red blood cells, white blood cells, proteins, and sugars in the urine.

Are there treatment options for Langerhans cell histiocytosis?

Prognosis is based on the stage of the disorder and the treatment provided. The prognosis also depends on the child’s general health and age. Your doctor may prescribe the following treatment options:

  1. steroid: Your child may be treated with steroids to suppress their immune system. However, you may need additional treatments, such as: chemical treatmentlow-dose radiation, or surgery.
  2. chemical treatment: In some cases, doctors may treat the disorder with chemotherapy. Certain forms of Langerhans cell histiocytosis resolve without treatment.
  3. surgery After chemotherapy to destroy lesions developed by the disorder
  4. UV therapy For the treatment of all skin diseases
  5. in severe casesorgan and tissue transplantation, lung, liver, bone marrow, etc.


Most people with LCH make a full recovery when treated. Unfortunately, patients who fall into the category of high-risk LCH involving the spleen, liver, or bone marrow have an 80% survival rate.

Frequently Asked Questions (FAQ)

What is another name for Langerhans cell histiocytosis?

Langerhans cell histiocytosis is also known as Hashimoto-Pritzger disease, histiocytosis X, Langerhans cell granulomatosis, and LCH.

Is Langerhans cell histiocytosis hereditary?

About 50% of people with this disorder have a gene that does not function properly, resulting in overproduction of Langerhans cells. This mutation occurs after birth. So there is very little inheritance from parent to child. Langerhans cell histiocytosis: symptoms, causes and treatment

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