Langerhans Cell Histiocytosis

Essential Information

Summarize

Langerhans cell histiocytosis (LCH) is a rare blood cancer caused by the abnormal proliferation of Langerhans cells, a type of white blood cell. LCH can occur at any age but is most common in young children. In children under 15 years old, the annual incidence of LCH is approximately 2 to 10 cases per 1 million children, with an average age of 2.5 years (median), and the ratio of males to females is nearly equal. The treatment for LCH in children differs from that for adults.

 

 

        LCH can cause damage to one or more organs and tissues. In medicine, the risk of LCH patients is usually classified according to the location of hyperplasia. Liver, spleen and hematopoietic system are high-risk cases, which are often accompanied by higher mortality rates.

Epidemiological

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Etiology & Risk Factors

Normal Langerhans cells (LC) are dendritic cells that fight infection. In LCH patients, LC cells develop genetic mutations (including mutations in the BRAF, MAP2K1, RAS, and ARAF genes), which cause rapid cell growth and proliferation, leading to accumulation of LC in certain tissues and organs and tissue damage.

 

 

 

        Risk factors for LCH include:

 

 

 

Parents are exposed to certain chemicals

Parents are exposed to metals, granite or wood chips at work

A family history of cancer, including LCH disease

A personal or family history of thyroid disease

The newborn had an infection

Smoking, especially among young people

Latino

Children who are not vaccinated and have more infections

Classification & Staging

There is no staging system for Langerhans cell histiocytosis (LCH). The severity or extent of the cancer is usually divided into different stages.

 

 

 

        The LCH treatment regimen is based on the location of the LCH cells found in the body and whether the LCH appears in a low-risk or high-risk organ.

 

 

 

        The difference between a single system or multiple systems of LCH disease is the number of body systems affected:

 

 

        

 

Single-system LCH: LCH is found in only one organ or body system. It may be a part of it or multiple components of the organ or system. Skeletal LCH is the most common single-system LCH disease.

 

Multi-system LCH: LCH occurs in two or more organs or systems, or may spread to the whole body. Multi-system LCH is less common than single-system LCH.

 

Recurrent LCH: LCH often recurs one year after treatment is stopped. It may recur in the same area, but it can also affect other parts of the body. It most commonly occurs in the bones, ears, skin or pituitary gland.

Clinical Manifestations

The most common manifestations of LCH are bone injuries with pain, followed by skin injuries. Clinical manifestations depend on the location of the lesion and whether multiple organ systems are involved. Symptoms may include fever, weight loss, diarrhea, edema, dyspnea, polydipsia, and polyuria.

 

 

        The symptoms of LCH depend on the organ where the damage occurs. When the disease occurs in certain specific organs, the patient's risk of death increases. These organs are called high-risk organs. The rest may develop but usually have good treatment outcomes are called low-risk organs.

 

 

 

        Low-risk organs include the skin, bones, lungs, lymph nodes, gastrointestinal tract, pituitary gland, thyroid, thymus, and the central nervous system (CNS), excluding the kidneys and reproductive glands. Children may develop a single organ disorder (single-system LCH) or multiple organ involvement due to tumor spread (multiple-system LCH). The condition can affect a single area (single focus) or multiple areas (multiple focus). While chronic and recurrent involvement of low-risk organs typically does not pose an immediate threat to life, it can lead to organ dysfunction.

 

 

 

        High-risk organs include the liver, spleen and hematopoietic system, defined by the presence of pathological LCH cells in the bone marrow. High-risk patients are usually younger than 2 years of age.

 

 

 

        The following symptoms may be caused by LCH, but they can also be caused by other causes. If you or your child has any of the following, please consult your doctor:

 

 

 

1. Manifestations of single system diseases

 

 

        Single-system diseases refer to the proliferation of cells in a single site or organ, including skin and nails, mouth, bones, lymph nodes and thymus, pituitary and thyroid.

 

 

 

        1.1 Skin and nails

 

 

 

        LCH may affect only the skin of infants, but it can also manifest as a multisystem LCH disease. In infants under one year old, LCH on the skin may resolve on its own, and treatment is typically only necessary for those with extensive rashes, pain, ulcers, or bleeding. However, these children require close monitoring, as LCH in newborns and infants can progress to a high-risk multisystem condition within weeks or months, which can be life-threatening.

 

 

 

        1.1.1 The following signs or symptoms may indicate LCH in infants:

 

 

 

The peeling of the scalp looks like eczema

Peeling in skin folds, such as inner elbows or perineum

A raised brown or purple rash on any part of the body

 

 

        1.1.2 In children and adults, LCH appearing on the skin and nails may present with the following signs or symptoms:

 

 

 

The scalp may appear to be flaking like dandruff

A red or brown rash may appear in the groin, abdomen, back or chest and may be accompanied by itching or pain

A bump or ulcer on the scalp

Ulceration behind the ear, under the breast or in the groin area

Nails may fall off or have discolored grooves running through them

 

 

         1.2 Mouth

 

 

 

          If LCH appears in the mouth, it may have the following signs or symptoms:

 

 

 

Gums swollen

Ulcers on the top of the mouth, inside the cheek, tongue or lips

Uneven or falling teeth are signs of bone involvement in the alveolar bone

 

 

         1.3 Skeletal system

 

 

 

         If LCH is present in the bone, there may be the following signs or symptoms:

 

 

 

Bone swelling or a lump on the bone, such as the skull, jawbone, ribs, pelvis, spine, thigh bone, upper arm bone, elbow, eye socket or bone around the ear ​

Bone pain

The risk of diabetes insipidus and other central nervous system disorders is increased when lesions occur in the bones around the ears or eyes

 

 

         1.4 Lymph nodes and thymus

 

 

 

         If LCH is present in the lymph nodes or thymus, the following signs or symptoms may be present:

 

 

 

 lymphadenectasis

 expiratory dyspnea

Superior vena cava syndrome. This can cause coughing, difficulty breathing, and swelling in the face, neck and upper arms

 

 

        1.5 Endocrine system

 

 

 

        1.5.1 Signs or symptoms affecting the pituitary gland due to LCH may include:

 

 

 

Diabetes insipidus. This causes intense thirst and frequent urination

 poor growth

Adolescence starts earlier or later

 overweight

 

 

         1.5.2 Signs or symptoms affecting the thyroid gland due to LCH may include:

 

 

 

The thyroid is swollen

Hypothyroidism can lead to fatigue, a lack of energy, sensitivity to cold, constipation, dry skin, thinning hair, memory problems, difficulty concentrating, and depression. In infants, it can also result in decreased appetite and food choking. In children and adolescents, it can cause behavioral and psychological issues, weight gain, growth delays, and late puberty

 expiratory dyspnea

 

 

         1.6 Eyes

 

 

 

         Signs or symptoms of LCH affecting the eye may include changes in vision, and even blindness.

 

 

 

         1.7 Central Nervous System (CNS)

 

 

 

         Signs or symptoms of LCH affecting the CNS may include:

 

 

 

Balance disorders, uncoordinated body movements, difficulty walking

 alalia

 hypopsia

 headache

Changes in behavior or personality

 allomnesia

 

           These signs and symptoms may be caused by

 

CNS

 

Lesion or

 

CNS

 

Caused by neurodegenerative syndrome.

 

 

 

 

 

        1.8, liver and spleen

 

 

 

         Signs or symptoms of LCH affecting the liver or spleen may include:

 

 

 

Abdominal swelling caused by an accumulation of extra fluid

 expiratory dyspnea

The skin turns yellow and the eyes turn white

 pruritus

Prone to bruising or bleeding

Easy to be tired

 

 

        1.9 Lungs

 

 

 

         Signs or symptoms that may affect lung LCH include:

 

 

 

Lung collapse. This condition can cause chest pain or tightness, difficulty breathing, feeling tired and blue skin

Breathing difficulties, especially in adult smokers

 dry cough

 pectoralgia

 

 

        1.10 Bone marrow

 

 

 

         Signs or symptoms that may affect bone marrow LCH include:

 

 

 

Prone to bruising or bleeding

 give out heat

Frequent infections

 

 

2. Manifestations of multiple system diseases

 

 

 

        In multisystem LCH, the disease affects multiple organs or systems, including bone, abdominal/gastrointestinal system (liver and spleen), lung, bone marrow, endocrine system, eye, central nervous system (CNS), skin, and lymph nodes. It is divided into high-risk sites (liver, spleen, bone marrow) and low-risk sites (all other sites).

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

 

        The disease is mainly based on X-ray and pathological examination results, and the key to diagnosis lies in the histological infiltration of Langerhans cells found in pathological examination. Therefore, biopsy should be done as far as possible.

 

 

 

        All possible tests are listed below.

 

      

2. Targeted inspection

 

      

 

BRAF gene test: a laboratory test for BRAF gene mutation in blood or tissue samples.

 

 

Water deprivation test: Check how much urine is produced and whether there is a test for urine concentration when little or no water is ingested. This test is used to diagnose diabetes insipidus that may be caused by LCH.

 

Bone marrow aspiration and biopsy: After local anesthesia, a bone marrow needle is inserted into the patient's hip to extract bone marrow and a portion of bone tissue. Pathologists examine the bone and bone marrow samples under a microscope to diagnose LCH. In most LCH patients, bone marrow proliferation is normal; however, in some cases, it may be hyperactive or hypoplastic, with signs of bone marrow invasion, anemia, and thrombocytopenia. Therefore, this test is only performed if peripheral blood abnormalities are detected.

 

Pulmonary function test (PFT): This test can measure how much air the lungs can hold and how fast air moves in and out of the lungs. It can also measure how much oxygen is used and how much carbon dioxide is emitted during breathing. LCH Pulmonary function examination: Patients with severe lung lesions may have varying degrees of pulmonary insufficiency, which often has a poor prognosis.

 

Bronchoscopy: This is a method to examine the abnormal areas of the trachea and airways within the lungs. The bronchoscope is inserted through the nose or mouth into the trachea and lungs. It is a thin, tubular instrument equipped with a light and a viewing lens. It may also have a tool for removing tissue samples, which are examined under a microscope for signs of cancer.

 

Endoscopy: This is a method to examine the internal organs and tissues of the body, particularly to identify abnormal areas in the gastrointestinal tract or lungs. The endoscope is inserted through an incision in the skin or an opening in the body (such as the mouth). It is a thin, tubular instrument equipped with light and a lens for observation. It may also have a tool to remove tissue or lymph node samples for microscopic examination of disease signs.

 

Biopsy: Cells or tissue are removed so that a pathologist can examine LCH cells under a microscope. To diagnose LCH, a biopsy can be performed from bone, skin, lymph node, liver or other diseased sites.

  

        The following tests can be performed on the tissue samples removed:

 

 

 

Immunohistochemistry: A technique for detecting antigens in tissue samples. Immunohistochemistry is often used to distinguish between types of cancer.

 

Flow cytometry: A laboratory test that measures the number of cells in a sample, cell survival rates, and cell size. It also measures the shape of the cells and whether tumor markers are present on the surface of the cells.

 

 

3. General examination

 

 

 

Physical examination and medical history: Examine the general condition of the body, including signs related to the disease, such as lumps or any visible abnormalities. Also ask about the patient's health habits, medical history, family history, and treatments received.

 

Neurological examination: Through inquiry and physical examination of brain, spinal cord and nerve function. Check the patient's mental state, coordination ability and normal walking ability; as well as muscle, sensory function and the good degree of neurological reflexes. This can also be called neurological examination.

 

Complete blood cell count (CBC): A blood sample is drawn to test for hemoglobin concentration, hematocrit, white blood cell differential count, red blood cell count, and platelet count. In LCH, routine blood tests often show no specific abnormalities; however, patients with multiple organ involvement frequently experience moderate to severe anemia, which may be accompanied by a decrease in white blood cells and a reduction in platelets.

 

Blood biochemistry: Tests for chemicals in blood samples (such as ferritin, triglycerides, fibrinogen, D-dimer, and lactate dehydrogenase) to see how well a patient's organs and tissues are functioning. Abnormal levels of substances in blood samples (above or below normal levels) may be a sign of disease.

 

Liver function tests: Used to measure the levels of chemicals (albumin, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and prothrombin time/particular clotting time) released by the liver in the blood. High or low levels of these substances can be a sign of liver disease.

 

Urine analysis: This involves examining the color and content of urine, including sugar, protein, red blood cells, white blood cells, etc. LCH urine specific gravity measurement: If the urine specific gravity is typically between 1.001 and 1.005, or if the urine osmolality is less than 200 mOsm/L, it may indicate damage to the pituitary gland or hypothalamus due to dihydropteridine destruction and tissue cell infiltration.

 

 

3. Imaging examination

 

        

 

        Lung X-ray examinations often reveal a reticular or reticular-like pattern of shadows in the lungs, with indistinct edges and not arranged according to the bronchial branches. Some lung fields may appear ground-glass, but in most cases, lung transparency increases, and small cystic bullae are common. In severe cases, the lungs may exhibit a honeycomb appearance, often accompanied by interstitial emphysema, mediastinal emphysema, subcutaneous emphysema, or pneumothorax. Many patients also develop pneumonia. At this stage, pulmonary cystic changes are more likely to occur. After the pneumonia subsides, the cystic changes may resolve, but the reticular and granular changes become more pronounced. Long-term patients may develop pulmonary fibrosis.

 

 

 

        Other commonly used imaging techniques are CT scans, magnetic resonance imaging (MRI), PET scans, ultrasound and bone scans.

 

 

 

 

Clinical Management

There are a number of treatments for LCH. Some are standardized and widely used, while others are currently under clinical trial.

 

 

 

 

The treatment plan for LCH patients is based on two considerations:

 

 

 

Whether it involves high-risk organs;

LCH is a single focus, multiple focus, or multiple system disease.

 

 

1. Standard therapy

 

        The current standard treatment for LCH includes nine: chemotherapy, surgery, radiotherapy, photodynamic therapy, immunotherapy, targeted therapy, other drug therapy, stem cell transplantation, and observation.

 

 

 

1.1 Chemotherapy

 

        LCH chemotherapy can be administered by injection, oral administration or other systemic chemotherapy, or it can be applied locally to the affected skin for local chemotherapy.

 

 

 

        Chemotherapy involves using chemical drugs to kill cancer cells or prevent their division, thereby inhibiting the growth of cancer cells. There are two types of chemotherapy: systemic and local. Systemic chemotherapy involves administering drugs orally or through intravenous or intramuscular injection into the bloodstream, which can reach cancer cells throughout the body. Local chemotherapy typically involves applying the drug directly to the skin or injecting it into the cerebrospinal fluid, organs, or body cavities (such as the abdomen), targeting and eliminating cancer cells in specific areas.

 

 

 

1.2 Surgery

 

The surgical removal of LCH lesions and a small amount of nearby healthy tissue is performed. Curettage involves using a curette (a sharp spoon-shaped tool) to scrape out LCH cells from the bone. In cases of severe liver or lung damage, the entire organ may need to be removed for transplantation. This requires finding a compatible donor to provide a healthy liver or lung.

 

 

 

1.3 Radiotherapy

 

        In LCH, special lamps can be used to send ultraviolet B (UVB) radiation to LCH skin lesions.

 

 

1.4 Photodynamic therapy

 

        Photodynamic therapy is a method of killing cancer cells using a drug and a type of laser. In photodynamic therapy called psoralen and ultraviolet A (PUVA) therapy, the patient receives a drug called psoralen and then ultraviolet A radiation directed at the skin.

 

 

 

After being injected intravenously, the drug is inactive until it is exposed to laser light. The drug is more concentrated in cancer cells than in normal cells. In LCH photodynamic therapy, the laser targets the skin, activating the drug and killing cancer cells. Photodynamic therapy causes minimal damage to healthy tissue. Patients undergoing photodynamic therapy should avoid sun exposure.

 

 

 

1.5 Immunotherapy

 

        Immunotherapy is a type of treatment that uses the patient's immune system to fight cancer. There are different types of immunotherapy:

 

Interferon treatment of skin LCH

Thalidomide for LCH

Intravenous immunoglobulin (IVIG) was used to treat neurodegenerative syndrome of central nervous system

 

 

Targeted therapy

 

        Targeted therapy is a treatment method that uses drugs or other substances to target and attack LCH cells without harming normal cells. Imatinib Mesylate, a tyrosine kinase inhibitor, is a targeted therapy drug. It prevents blood stem cells from transforming into dendritic cells, which can potentially mutate into cancer cells. Currently, other types of kinase inhibitors, such as vemurafenib, which targets BRAF gene mutations, are also being tested in clinical trials for LCH.

 

 

 

        Mutations in genes within the RAS gene family can lead to cancer. The RAS protein is involved in cell signaling, growth, and death. RAS pathway inhibitors are targeted therapies under clinical trial investigation. These inhibitors block the function of mutated RAS genes or proteins, potentially preventing cancer growth.

 

 

 

1.7 Other drug therapy

 

        Other drugs used to treat LCH include:

 

Steroids: such as prednisone, are used to treat LCH lesions

Bisphosphonates: such as pamidronate, zoledronic acid or alendronate are used to treat LCH lesions of the bone and relieve bone pain

Anti-inflammatory drugs: such as pioglitazone and rofecoxib, are used to reduce fever, swelling, pain and redness. Anti-inflammatory drugs can be given concurrently with chemotherapy drugs to treat adult bone LCH

Vitamin A-like: such as isotretinoin, which slows the growth of LCH cells in the skin. Vitamin A-like is an oral drug

 

 

1.8 Stem cell transplantation

 

Stem cell transplantation involves administering chemotherapy and simultaneously transplanting stem cells to replace the blood-forming cells damaged by chemotherapy. Stem cells, which are immature blood cells, are extracted from the patient's or donor's blood or bone marrow and stored in a frozen state. After chemotherapy, the stored stem cells are thawed and infused back into the patient's body. These reinfused stem cells help restore the body's blood cell production.

 

 

 

1.9 Observation

 

Observation involves closely monitoring the patient's condition without giving any treatment until signs or symptoms appear or change.

 

 

 

2. Clinical trials

 

 

Clinical trials are part of the cancer research process. They are conducted to determine whether new cancer treatments are safe and effective or better than current standard therapies. Many of today's standard cancer treatments are based on early clinical trials.

 

 

 

For some patients, participating in a clinical trial may be the best treatment option. Patients who participate in a clinical trial may be offered standard care or may be one of the patients receiving a new therapy.

 

 

 

The ongoing clinical trials in the United States are available on the ClinicalTrials.gov website.

 

 

 

3. Specific treatment of LCH in children

 

 

 

3.1 Treatment of low-risk diseases in children

 

3.1.1 Treatment of cutaneous manifestations of Langerhans cell histiocytosis (LCH) includes:

 

 

 

(1) Observation.

 

 

 

(2) When severe rash, pain, ulceration or bleeding occurs, treatment may include:

 

Steroid therapy

Chemotherapy is given orally or intravenously

Skin administration of chemotherapy

Photodynamic therapy was performed with psoralen and ultraviolet A (PUVA) therapy

Ultraviolet B (UVB) radiation therapy

 

 

3.1.2 Lesions of bones or other low-risk organs

 

 

 

Depending on the location of the diseased bone or organ, the treatment plan may vary slightly.

 

 

 

(1) If the lesion occurs in the anterior, lateral or posterior cranial bone, the treatment plan usually includes:

 

Surgical treatment (curettage) may be supplemented with steroid therapy

Low-dose radiation therapy is usually used for lesions that have affected nearby organs

 

 

(2) If the child's LCH lesion is in the bone around the ear or eye, treatment may be needed to reduce the risk of diabetes insipidus or other long-term disease. Treatment may include:

 

Chemotherapy and steroid treatment

Surgery (curettage)

 

 

(3) If the child's LCH lesion is located in the spine or femur, treatment may include:

 

 observe

Low-dose radiotherapy

Chemotherapy is used when the lesion has spread from the spine to nearby tissues

The weakened bone is strengthened by osteoplastic or osteoinductive surgery

    

 

(4) If the lesion occurs in two or more bones, the treatment plan usually includes:

 

Chemotherapy and steroid treatment

      

 

(5) If the lesion occurs in two or more bones, accompanied by skin lesions, lymph node lesions or diabetes insipidus, the treatment plan usually includes:

 

Chemotherapy may be supplemented with steroid therapy

Denosumab treatment

 

 

        3.1.3 Central nervous system lesions

 

 

 

(1) Treatment options for LCH lesions of the central nervous system (CNS) in children may include:

 

Chemotherapy may be supplemented with steroid therapy.

   

 

(2) Treatment of neurodegenerative diseases caused by LCH may include:

 

Treatment with retinoid analogs

Immunotherapy (IVIG) may be supplemented with chemotherapy

 chemotherapy

 targeted therapy

 

 

3.2 Treatment of high-risk diseases in children

 

 

        If LCH is a multisystem disease affecting the spleen, liver or bone marrow and other organs, the treatment may include:

 

Chemotherapy and steroid therapy. For patients with tumors that do not respond to initial chemotherapy, higher doses of multiple chemotherapeutic agents can be given in combination with steroids

Liver transplantation was performed in patients with severe liver injury

Clinical trials are tailored to the patient's condition based on the characteristics of the cancer and how it responds to treatment

Clinical trials of chemotherapy and steroid therapy

 

 

3.3 Treatment regimens for recurrent, refractory and progressive LCH in children ​

 

 

        Recurrent LCH refers to cancer that disappears after treatment but then recurs. Refractory LCH refers to cancer that does not respond well to treatment. Progressive LCH refers to cancer that continues to deteriorate during treatment.

 

 

 

        3.3.1 Treatment of relapsed, refractory or progressive low-risk LCH may include:

 

 

 

Chemotherapy may be supplemented with steroid therapy

Denosumab treatment

 

 

        3.3.2 Treatment of relapsed, refractory or progressive high-risk multisystem LCH may include:

 

 

 

 high dose chemotherapy

Stem cell transplantation

 

 

        3.3.3 Clinical trials currently under investigation for recurrent, refractory or progressive LCH in children include:

 

 

 

Tailor the patient's treatment to the characteristics of the cancer and how it responds to treatment

Genetic testing of the patient's tumor samples was performed and a matched targeted treatment regimen was given to the patient

Clinical trials of targeted therapy drugs (vemurafenib or imatinib)

 

 

        Tip:

 

        The information provided by Sunflower Children's is for reference only and cannot replace the advice of doctors and other medical personnel, nor can it replace the doctor's face-to-face consultation. If you have specific questions about disease, treatment plan, diagnosis or clinical symptoms, please seek help from professional medical personnel.

 

 

 

 

Prognosis

LCH that occurs in the skin, bones, lymph nodes or pituitary gland usually has a better prognosis and is called a "low-risk" organ. LCH that occurs in the spleen, liver or bone marrow is more difficult to treat and is called a "high-risk" organ.

 

      The prognosis of LCH depends on:

 

Age at which the child was diagnosed with LCH

50. Which organ or organs/systems are affected by LCH
50. How many organs or systems are affected by cancer
50. Cancer can occur in certain bones, such as the liver, spleen, bone marrow or skull
50. Response to initial treatment of cancer
50. Whether there is a mutation in the BRAF gene
50. Whether it's a first diagnosis or a recurrence

 

        In infants under one year of age, LCH may disappear without treatment, but it still needs to be closely monitored.

 

1. Recurrence rate

 

      Many children with LCH (Langerhans cell histiocytosis) show improvement after treatment, but they often experience a relapse or the appearance of new lesions after treatment is discontinued. Recurrence typically occurs within a year after treatment cessation. The recurrence rate for monosystem LCH is 9%-17.4%; for monosystem multifocal disease, it is about 37%; for multisystem low-risk LCH, the recurrence rate is 46%, and for high-risk LCH, it can be as high as 54%. Common sites of recurrence include bones, ears, or skin, and diabetes insipidus may also occur. Less common sites of recurrence include lymph nodes, bone marrow, spleen, liver, or lungs.

 

​

      

      The average recurrence time (median) is 9 months (for high-risk patients) and 12-15 months (for low-risk patients). About one-third of patients have multiple recurrences between 9 and 14 months after treatment. Recurrence rates from multiple clinical trials are roughly the same.

 

      Because of the risk of recurrence, children with LCH should insist on follow-up. During follow-up, some of the tests used to diagnose LCH may be repeated to monitor the effectiveness and to detect new lesions. These tests may include:

 

50.  general inspection
50.  neurological examination
50. Imaging studies
50. Brainstem auditory evoked response (BAER) test: A test that examines the brain's response to clicking sounds or certain tones
50. Pulmonary function tests (PFT)
50. Chest X-ray

        

      The results of these tests can show whether the child's condition has changed or if cancer has returned. The results help determine whether to continue, change or stop treatment.

 

2. After-effects

 

      Children with low-risk single-system LCH (skin, bone, lymph node, or pituitary) have about a 20% chance of developing long-term sequelae; for those with multiple systems, this rate rises to 70%. The most common long-term sequelae include hypothalamic/pituitary dysfunction (50%), cognitive impairment (20%), and cerebellar damage (17.5%). These sequelae may affect the following bodily systems:

 

2.1, endocrine

 

The child has the risk of total pituitary dysfunction, and the growth and development of the child should be carefully monitored;

The child may have a growth hormone deficiency, which can affect growth and development.

 

2.2 Auditory system

 

LCH treatment may lead to hearing loss.

 

      2.3 Nervous system

 

50. Children with spinal lesions may have symptoms of nerve system compression;
50. Children with central nervous system LCH may have major cognitive defects, cerebellar dysfunction and behavioral abnormalities, short-term memory loss and other sequelae.

 

      2.4 Skeletal system

 

50. Lesions of spine, femur, tibia or humerus;
50. Including vertebral collapse or spinal instability. May lead to scoliosis, facial or limb asymmetry, etc.

 

      2.5 Breathing

 

50. Diffuse lung disease can lead to weakened lung function, increased risk of infection, and reduced exercise tolerance. These children should be examined for lung function.

 

        2.6 Digestion

 

50. Liver disease can lead to sclerosing cholangitis, which usually requires a liver transplant;
50. Some children may have dental problems after tooth surgery if they need to be treated with LCH.

 

       2.7 Secondary tumors

 

50. Secondary bone marrow failure following LCH or LCH treatment is rare but usually associated with high-risk malignancies. The risk of secondary cancer in children with LCH is increased.

 

Follow-up & Review

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Daily Care

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Cutting-edge therapeutic and clinical Trials

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References

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Audit specialists

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