Langerhans Cell Histiocytosis

Summarize

1. General remarks

● Overview: Langerhans cell histiocytosis is common in children, with a peak age of 1-4 years.

● Manifestation: The clinical manifestations vary greatly. Mild cases only show self-limiting simple bone and skin lesions, while severe cases can be fatal systemic multi-organ or multi-system lesions.

● Treatment: The clinical manifestations of Langerhans cell histiocytosis are complex and varied, and the treatment plan should be adjusted according to the lesion site and range [2]. Common treatments include chemotherapy, surgery and radiotherapy.

● Prognosis: The prognosis of adults is better than that of children, and the prognosis of single-system type is better than that of multiple system type. The 10-year survival rate of adults reaches 86%. [3] The 3-year overall survival rate of high-risk group of children is about 72%, and the 3-year overall survival rate of low-risk group of children is 100%.

2. Disease definition

Langerhans cell histiocytosis (LCH), commonly known as 'Langhans' disease, is a rare histiocytic disorder. It was formerly referred to as 'histiocytosis X.' The disease is characterized by the abnormal accumulation of large numbers of pathological Langerhans cells, which are immune cells that help fight infections, in tissues. It is currently believed to be an inflammatory myeloid tumor.

Epidemiological

。 epidemiology

The annual incidence of Langerhans cell histiocytosis is estimated to be 0.5~5.4 per 100,000 people, with slightly more males. Langerhans cell histiocytosis can be seen in any age group, commonly in children, the peak age of onset is 1-4 years old, and the incidence in adults is low.

In people aged 15 and under, the annual incidence of Langerhans cell histiocytosis is 0.2 to 1 per 100,000, with a median age of onset of 30 months. The annual incidence of Langerhans cell histiocytosis in adults is estimated at 1 to 2 per million.

Etiology & Risk Factors

1. General remarks

Langerhans cell histiocytosis originates from pathological Langerhans cells. These cells, which are immune cells that fight infections, may undergo genetic mutations during their development, such as mutations in genes like BRAF, MAP2K1, RAS, or ARAF. These mutations can cause Langerhans cells to grow rapidly and accumulate in specific areas of the body, leading to tissue damage and the formation of space-occupying lesions. However, the exact cause of this disease remains unclear.

2. Basic etiology

Currently, over 50% of patients with Langerhans cell histiocytosis have BRAF V600E mutations in their affected tissues. Among patients without BRAF V600E mutations, 33% to 50% have mutations in the MAP2K1 gene or other genes in the MAPK signaling pathway, such as mutations in the ARAF gene or ERBB3 gene. These genetic mutations can occur at various stages of hematopoietic cell development. If they occur during the early bone marrow stem cell stage, the disease is often clinically characterized by high-risk involvement of multiple systems. If the mutations occur during the later Langerhans cell stage, the disease is typically low-risk and affects a single system. Therefore, Langerhans cell histiocytosis is currently considered a hematological tumor primarily characterized by the activation of the MAPK signaling pathway, classified as an inflammatory myeloid tumor.

3. Triggering factors

Currently, the exact causes of Langerhans cell histiocytosis are not fully understood. Some studies have identified factors associated with the onset in children, such as parental exposure to certain chemical solvents, a family history of cancer, personal or familial thyroid disease, perinatal infections, parental occupational exposure to dust (including metal dust, granite dust, or wood dust), ethnic and racial factors (such as a higher prevalence among Hispanic children), and a lack of childhood vaccinations. However, whether these factors can trigger Langerhans cell histiocytosis requires further research.

Classification & Staging

Type of illness

1) Disease classification

Langerhans cell histiocytosis is classified as single system type and multiple system type according to the number of affected systems.

Single-system Langerhans cell histiocytosis (single-system LCH, SS-LCH) is a disease affecting a single organ or system. According to the number of affected sites, it can be divided into single-site and multiple site types. Bone is the most common site of single-site Langerhans cell histiocytosis.

Multisystem LCH (MS-LCH): Multiple organs or systems are affected and may spread throughout the body. Multisystem lesions are less common than single system lesions.

2) Disease grouping

According to the non-segmentation of Langerhans cell histiocytosis, it is divided into low-risk group and high-risk group only according to whether it involves high-risk organs.

● Low-risk group: the affected organs include skin, bone, lung, lymph node, gastrointestinal tract, pituitary gland, thyroid, thymus and central nervous system.

● High-risk group: affected organs include liver, spleen and bone marrow.

Clinical Manifestations

1. General remarks

The clinical manifestations of Langerhans cell histiocytosis vary greatly among different patients. Mild cases only have self-limiting simple bone or skin lesions, which can heal by themselves without special treatment; severe cases may be manifested as serious systemic multi-organ or multi-system lesions, which may endanger life.

The most common clinical manifestations in children are painful bone space, followed by skin symptoms, and some systemic symptoms such as fever, weight loss, diarrhea, edema, dyspnea, thirst and polyuria, which may be related to the involvement of specific organs or to the presentation of a single or multiple systems.

The clinical presentation in adults is similar to that in children. All organs or systems affected in children are also affected in adults. However, the incidence of specific organ involvement differs between adults and children. The most frequently affected organs in adults are the lungs (usually a single-system disease and closely associated with smoking), followed by bones and skin.

2. Typical symptoms

1) Skin and nail symptoms

Infant Langerhans cell histiocytosis may affect only the skin, but some infants with only skin symptoms may progress to high-risk multisystem Langerhans cell histiocytosis over several weeks or months. Symptoms of infant skin involvement include: scalp lesions resembling milia, skin damage in body folds (such as the elbow crease and perineum), and brown or purple raised rashes.

Symptoms of nail issues in both children and adults include: flaking skin similar to dandruff, red or brown raised rashes on the groin, abdomen, back, or chest (which may be itchy or painful), scalp lumps or ulcers, skin ulcers behind the ears, under the breasts, or in the groin area, and nail loss or transverse discoloration grooves on the nails.

2) Oral symptoms

Oral symptoms include swollen gums, ulcers in the palate, misaligned teeth or tooth loss.

3) Bone involvement symptoms

Symptoms of bone involvement include: Swelling or lumps around the bone (such as the skull, jawbone, ribs, pelvis, vertebrae, femur, humerus, elbow, eye socket or ear) and pain from swelling or lumps around the bone.

4) Symptoms of lymph node and thymus involvement

Symptoms of lymph node and thymus involvement include swollen lymph nodes, difficulty breathing, superior vena cava syndrome (which can cause cough, difficulty breathing, swelling of the face, neck and upper arm).

5) Endocrine system symptoms

The disease can affect the brain's pituitary gland and thyroid gland, causing a range of endocrine system symptoms.

Symptoms caused by pituitary involvement include: diabetes insipidus (causing intense thirst and frequent urination), growth retardation, precocious or late puberty, and severe overweight.

Symptoms caused by thyroid involvement include: an enlarged thyroid, hypothyroidism (which can manifest as fatigue, weakness, cold intolerance, constipation, dry skin, thinning hair, memory issues, difficulty concentrating, and depression; infants may have a lack of appetite and difficulty swallowing; children may experience delayed growth and sexual development; both children and adults may exhibit abnormal behavior, weight gain), and breathing difficulties.

6) Eye symptoms

The eyes can suffer and may cause vision problems.

7) Central nervous system symptoms

Symptoms caused by involvement of the central nervous system (including the brain and spinal cord) may include: loss of body balance, uncoordinated body movements and difficulty walking, speech difficulties, visual problems, headaches, behavioral or personality changes, and memory problems.

8) Symptoms of liver and spleen involvement

Symptoms caused by liver or spleen involvement include abdominal distension due to fluid accumulation, difficulty breathing, jaundice of the skin and sclera, itching, easy bruising, easy bleeding, fatigue, etc.

9) Pulmonary symptoms

Lung involvement can cause lung collapse, resulting in chest pain, chest tightness, dyspnea, fatigue, bruising, and other symptoms. Other lung involvement symptoms include dyspnea (especially in adult smokers), dry cough, and chest pain.

10) Bone marrow involvement symptoms

Symptoms caused by bone marrow involvement include easy bruising, easy bleeding, fever, and frequent infections.

3. Accompanying symptoms

Involvement of the central nervous system (including brain and spinal cord) may cause central nervous system neurodegenerative syndrome, which may be similar to the manifestations of central nervous system involvement.

Clinical Department

1. General remarks

The main diagnostic basis for Langerhans cell histiocytosis is medical history, physical examination and histopathological examination.

2. Department of treatment

Langerhans cell histiocytosis can affect multiple organs, leading to a wide range of clinical symptoms. Depending on the affected organs or systems, patients may visit departments such as pediatric orthopedics, pediatric surgery, pediatric dermatology, pediatric oncology, pediatric hematology, pediatric endocrinology, pediatric gastroenterology, pediatric respiratory medicine, and pediatric ophthalmology. For adult patients, the departments they might visit include respiratory medicine, orthopedic surgery, dermatology, oncology, gastroenterology, and ophthalmology.

3. Diagnostic basis

Pathological diagnosis is the gold standard for diagnosing Langerhans cell histiocytosis.

4. Related checks

1) Blood routine

The results of blood routine in patients with Langerhans cell histiocytosis are usually not specific, and most of them have positive cell normochromic anemia of varying degrees. Thrombocytopenia may occur in severe patients. Only 1/6 of pediatric patients have eosinophils>4%.

2) Blood biochemistry

Patients with Langerhans cell histiocytosis with liver involvement may have elevated liver and bile duct enzymes, and in severe cases may have abnormal signs similar to cirrhosis. Patients with diabetes insipidus may have elevated blood sodium and lower urine osmolality than plasma osmolality.

3) Endocrine examination

If the pituitary gland is affected, growth hormone (GHD) deficiency, sex hormone deficiency, adrenocorticotropic hormone (ACTH) deficiency and thyroid-stimulating hormone (TSH) deficiency may occur.

4) Bone evaluation

Depending on the patient's condition, some patients may need bone evaluation. X-rays and CT scans (such as whole-body low-dose CT) are often the preferred examination methods. MRI and PET-CT (positron emission tomography-computed tomography) are also viable options, as they can detect bone lesions simultaneously and assess treatment outcomes.

5) Neurological examination

Depending on the needs of the patient, a series of tests are required to evaluate brain, spinal cord and nerve function, as well as mental state, body coordination, walking ability, and normal muscle tone, sensory nerves and reflexes.

6) Water prohibition test

Nodular histiocytosis may cause diabetes insipidus. Depending on the condition, some children need to do a water fast test to diagnose diabetes insipidus. The test allows patients to drink only a small amount of water or prohibit them from drinking water, and then tests their urine volume, urine specific gravity and urine osmolality.

7) Bone marrow aspiration

Depending on the needs of the disease, some patients need to do bone marrow puncture (bone marrow puncture for short), followed by immunohistochemistry and/or flow cytometry to check cell antigens and tumor markers, so as to help the diagnosis and typing of tumors.

8) Other histopathological biopsies

Depending on the need, some patients may require additional histopathological biopsies to help with diagnosis. Biopsy tissue may include bone, skin, lymph nodes, liver, or other sites of lesion.

9) Pulmonary examination

If there is lung involvement, a lung examination is required, usually through high-resolution CT. Pulmonary function and bronchoalveolar lavage fluid tests can also be performed.

10) Other imaging examinations

According to the needs, B-ultrasound can be used to examine thyroid masses in the neck and abdominal organs, as well as intracranial lesions. If the pituitary gland is suspected to be affected, MRI (magnetic resonance imaging) can be used for examination. PET-CT can be performed when necessary to evaluate the extent of systemic involvement of the disease.

5. Differential diagnosis

1) Differential diagnosis with sclerotic histiocytosis

Sclerosing histiocytosis is a rare non-Langerhans cell histiocytosis characterized by the progressive formation of fibrous and clonal histiocytes. It can be differentiated from Langerhans cell histiocytosis through pathological examination. In patients with sclerosing histiocytosis, both CD1a and CD207 are negative in the affected tissues, and electron microscopy reveals no characteristic Birbeck granules found in Langerhans cell histiocytosis, which helps to differentiate it.

However, some patients may have both diseases at the same time, and the pathological manifestations of both diseases can occur in a single lesion or in different organs or systems.

2) Differential diagnosis with undifferentiated dendritic cell tumors

Undifferentiated dendritic cell tumors are a rare type of tumor that originates from the precursor cells of Langerhans cells. These tumors can be differentiated from Langerhans cell histiocytosis through pathological examination. In patients with this condition, CD1a may be positive, while CD207 is negative. Additionally, electron microscopy reveals the absence of characteristic Birbeck granules found in Langerhans cell histiocytosis, which aids in the differential diagnosis.

Examination & Diagnosis

Diagnosis criteria

Pathological diagnosis is the gold standard for diagnosing Langerhans cell histiocytosis.

coherence check

1) Blood routine

2) Blood biochemistry

3) Endocrine examination

If the pituitary gland is affected, growth hormone (GHD) deficiency, sex hormone deficiency, adrenocorticotropic hormone (ACTH) deficiency and thyroid-stimulating hormone (TSH) deficiency may occur.

4) Bone evaluation

Based on the patient's condition, some patients may require bone evaluation. X-rays and CT scans (such as whole-body low-dose CT) are often the preferred examination methods. MRI and PET-CT (positron emission tomography-computed tomography) are also viable options, as they can detect bone lesions simultaneously and assess treatment outcomes.

5) Neurological examination

6) Water prohibition test

7) Bone marrow aspiration

Depending on the needs of the disease, some patients need to do bone marrow puncture (bone marrow puncture for short), and then do immunohistochemistry and/or flow cytometry to check cell antigens and tumor markers, so as to help the diagnosis and typing of tumors.

8) Other histopathological biopsies

Depending on the need, some patients may require additional histopathological biopsies to help with the diagnosis. Biopsies may include bone, skin, lymph nodes, liver, or other sites of lesion.

9) Pulmonary examination

If there is lung involvement, a lung examination is required, usually through high-resolution CT. Pulmonary function and bronchoalveolar lavage fluid tests can also be performed.

10) Other imaging examinations

antidiastole

1) Differential diagnosis with sclerotic histiocytosis

However, some patients may have both diseases at the same time, and the pathological manifestations of both diseases can occur in a single lesion or in different organs or systems.

2) Differential diagnosis with undifferentiated dendritic cell tumors

Undifferentiated dendritic cell tumors are a rare type of tumor that originates from the precursor cells of Langerhans cells. These tumors can be differentiated from Langerhans cell histiocytosis through pathological examination. In patients with this condition, CD1a may be positive, but CD207 is negative. Additionally, electron microscopy reveals the absence of characteristic Birbeck granules found in Langerhans cell histiocytosis, which aids in the differential diagnosis.

Clinical Management

1. General remarks

The treatment of Langerhans cell histiocytosis depends on whether the affected areas are in a single or multiple systems. For patients with a single system type, local treatments, such as targeted radiotherapy to the lesion, can be effective. In contrast, for patients with multiple system type, systemic treatments are primarily used, often involving chemotherapy drugs that target myeloid tumors. Patients with mutations like BRAFV600E can opt for targeted therapy.

At the same time, in neonatal Langhans cell histiocytosis and high-risk, multisystem, recurrent or refractory cases, related gene mutations are often associated, so early genetic testing can be considered to avoid overtreatment for precise treatment.

2. General treatment

The treatment of Langerhans cell histiocytosis should be based on the type and extent of the lesion:

1) Treatment of low-risk Langerhans cell histiocytosis in children

I) Treatment of skin lesions

If the child develops skin lesions, the following treatments can be used: steroid therapy, topical chemotherapy, systemic chemotherapy (oral or intravenous), photodynamic therapy (psoralen and ultraviolet A), and ultraviolet B irradiation.

ii) Treatment of bone lesions or other low-risk organ lesions

For primary bone lesions in children that occur on the anterior, posterior, or bilateral sides of the skull, or in other single bones, osteotomies can be used for treatment (with or without steroids). If the lesion invades nearby tissues and organs, low-dose radiation therapy may be considered.

For children with primary bone lesions in the ear or eye, chemotherapy combined with steroids and orthopedic curettage can be used to reduce the risk of diabetes insipidus.

For children with bone lesions in the spine or femur, which are usually treated with low-dose radiotherapy. If the spine lesion invades nearby tissues, chemotherapy is required. Surgery can also be used to support or fuse the damaged bone.

If the child has two or more bone lesions, chemotherapy combined with steroids is required.

If the child has two or more bone lesions and is also associated with skin lesions, lymphomas, or diabetes insipidus, chemotherapy (with or without steroids) and bisphosphonates are generally used.

iii) Treatment of central nervous system lesions

For children with central nervous system lesions treated for the first time, chemotherapy is usually used, with or without steroids.

If a child has central nervous system (CNS) lesions and is also suffering from CNS neurodegenerative syndrome, treatment options may include chemotherapy, retinoic acid therapy, intravenous immunoglobulin (IVIg), either in combination with or without steroids. Additionally, based on the patient's condition, targeted therapies such as BRAF inhibitors (vemurafenib, dabrafenib, trametinib) and rituximab can be considered.

2) Treatment of high-risk Langerhans cell histiocytosis in children

For children with high-risk Langerhans cell histiocytosis, initial treatment typically involves a combination of chemotherapy drugs and steroids. If the initial treatment is not effective, higher doses of chemotherapy drugs combined with steroids may be necessary. Targeted therapies such as vemurafenib, dabrafenib, and trametinib can also be considered. In cases where the child has severe liver damage, liver transplantation may be an option.

3) Treatment of recurrent or refractory pediatric Langerhans cell histiocytosis

If the patient with Langerhans cell histiocytosis (LCH) experiences a recurrence, or if the treatment is ineffective, or if the disease continues to progress during treatment, low-risk patients may be treated with chemotherapy (with or without steroids) and bisphosphonates. High-risk patients should undergo high-dose chemotherapy, which may include targeted therapies such as vemurafenib, dabrafenib, and trilaciclib, or autologous hematopoietic stem cell transplantation.

Since many cases of recurrent or refractory pediatric Langerhans cell histiocytosis have gene mutations such as BRAF V600E, early genetic testing can also be considered for children, and if available targeted drugs are available, they can be added to the treatment regimen to improve the likelihood of cure.

4) Treatment of adult Langerhans cell histiocytosis

In adult Langerhans cell histiocytosis, the most common pulmonary lesions are monosystemic, followed by bone or skin lesions.

Patients with lung lesions are usually treated with chemotherapy, and patients with severe lung damage may consider a lung transplant. Smokers must quit smoking, otherwise lung damage will continue to worsen.

Patients with bone lesions can be treated with surgery (with or without steroids), chemotherapy (with or without low-dose radiotherapy) or radiotherapy, and bisphosphonates for severe pain. Doctors may also consider anti-inflammatory treatment for patients.

Patients with skin lesions may consider the following treatment options: surgery, steroids or other medications (topical on the skin), photodynamic therapy (using psoralen and ultraviolet A), UV-B irradiation, chemotherapy (such as methotrexate, thalidomide, and hydroxyurea), or immunotherapy (interferon). If other treatments are ineffective for the skin damage, retinoid therapy can also be considered.

For other organ involvement in adults with single-system and multiple-system diseases, chemotherapy or targeted drugs such as imatinib or vemurafenib are usually used.

3. Chemotherapy

Chemotherapy drugs work by either killing tumor cells or preventing their division, thereby inhibiting tumor growth. Systemic chemotherapy is administered orally or through intravenous or intramuscular injection, allowing the drugs to enter the bloodstream and target tumor cells. Local chemotherapy involves direct application to the skin or injection into cerebrospinal fluid, specific organs, or body cavities (such as the abdominal cavity), targeting tumor cells in these areas. Commonly used chemotherapy drugs for Hodgkin's lymphoma include vincristine, cytarabine, etoposide, and cladribine.

4. Drug therapy

Langerhans cell histiocytosis is commonly treated with a combination of steroid hormones (such as prednisone) and other therapies to reduce side effects. Other drugs that can be used include bisphosphonates (such as alendronate, zoledronic acid, or alendronate sodium), anti-inflammatory drugs (such as pioglitazone and rofecoxib, which are typically used to reduce fever, swelling, and pain), and retinoids (such as isotretinoin, which can slow the growth of proliferating Langerhans cells in the skin).

5. Radiotherapy

Radiation therapy (RT) is a cancer treatment that uses high-energy X-rays or other types of radiation to kill cancer cells or prevent their growth. For patients with systemic Langerhans cell histiocytosis, some can benefit from local RT treatment. Additionally, some patients with bone lesions can also be treated with RT.

6. Surgical treatment

The lesion of Langerhans cell histiocytosis and a small part of the healthy tissue near the lesion are removed by surgical resection. For example, for bone lesions of Langerhans cell histiocytosis, osteotomies can be used to scrape off the lesion.

7. Other treatments

1) Photodynamic therapy

Photodynamic therapy is a novel cancer treatment that uses drugs (photosensitizers) and specific lasers to target and destroy cancer cells. Because tumor cells are highly metabolically active, the drug concentration in tumor tissues is significantly higher than in surrounding normal tissues after injection. When a laser of a specific wavelength is applied to the tumor tissue, it activates the photosensitizers, producing singlet oxygen ions that specifically destroy cancer cells and their new blood vessels. In the case of Langerhans cell histiocytosis, photodynamic therapy is used to treat skin lesions. For example, in the treatment with psoralen and ultraviolet A, patients are injected with psoralen and then exposed to ultraviolet A directly on the affected skin area. It is important to note that patients undergoing photodynamic therapy should avoid prolonged sun exposure.

2) Ultraviolet therapy

For cutaneous lesions of Langerhans cell hyperplasia, ultraviolet B irradiation can be used for treatment.

8. Frontier treatment

1) Targeted therapy

Targeted therapy uses drugs that specifically target tumor cells. Compared to chemotherapy or radiotherapy, targeted therapy causes less damage to normal cells. Several targeted therapy drugs are available for treating Langerhans cell histiocytosis, including tyrosine kinase inhibitors (imatinib), BRAF inhibitors (vemurafenib, dabrafenib, and trametinib), and monoclonal antibodies (rituximab).

Currently, targeted drugs are not the first-line treatment for Langerhans cell histiocytosis. However, case reports have shown their effectiveness in treating this condition. For high-risk, multisystem, recurrent, or refractory cases of Langerhans cell histiocytosis, early genetic testing can help identify potential targeted drugs.

2) Immunotherapy

Immunotherapy is a treatment that leverages the patient's immune system to fight cancer. Several immunotherapies are used to treat Langerhans cell histiocytosis, including interferon for skin lesions, thalidomide for the condition, and intravenous immunoglobulin for central nervous system disorders (a complication of Langerhans cell histiocytosis).

Prognosis

1. General remarks

Patients with Langerhans cell histiocytosis have a better prognosis in adults compared to children, and those with single-system involvement have a better prognosis than those with multiple-system involvement. The 10-year survival rate for adult patients is approximately 86%, with the primary permanent complication being diabetes insipidus due to pituitary involvement. In pediatric patients, the 3-year overall survival rate for high-risk cases is about 72%, and the 3-year event-free survival rate is about 46%; for low-risk cases, the 3-year overall survival rate is as high as 100%, and the 3-year event-free survival rate is about 82%. Infants with Langerhans cell histiocytosis are difficult to predict, with mild cases showing only skin lesions that can resolve on their own within a few months; severe cases can rapidly deteriorate, affecting multiple systems, leading to a high mortality rate.

2. Aftereffects

Depending on the site and extent of the lesion, some sequelae may occur after treatment, including slow growth and development, hearing loss, bone, tooth, liver and lung function problems, mood changes, learning and memory problems, and risk of other cancers (such as leukemia, retinoblastoma, Ewing's sarcoma, brain cancer, liver cancer).

Patients with partial multisystem Langerhans cell histiocytosis may experience some aftereffects, which could be due to the disease's progression or adverse reactions from treatment. These patients might face long-term health issues that can impact their quality of life. Some of these adverse reactions are treatable or controllable, so it is important to have a detailed discussion with your doctor about the initial treatment plan.

3. Recurrence

Most patients with Langerhans cell histiocytosis have good treatment outcomes, but new lesions may develop and old lesions may reappear for some time after treatment, known as disease recurrence. Recurrence may occur within a year of treatment.

Patients with multisystem lesions are more likely to have recurrence. Common sites of recurrence are bone, ear or skin. Less common but also occur sites include: lymph nodes, bone marrow, spleen, liver, and lungs. Some patients may have multi-site recurrence after several years.

For patients with high-risk or multisystem lesions, genetic testing can be considered early. If applicable targeted drugs are available, they can be added to the treatment regimen to reduce the likelihood of recurrence.

Follow-up & Review

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Daily Care

1. General remarks

After the end of treatment, patients need regular follow-up visits to detect recurrence and long-term effects.

2. Review

Because of the risk of recurrence, patients with Langerhans cell histiocytosis need to be monitored for a long time. The time and frequency of follow-up vary according to different disease types and groups.

The examination required for review includes: internal medicine examination, neurological examination, imaging examination (such as B ultrasound, magnetic resonance (MRI), CT, PET scan, etc.).

Depending on the patient's specific condition, other tests that may be required include: brainstem auditory evoked response (which examines the patient's brain's response to a click or a specific tone), pulmonary function tests, chest X-rays, etc.

3. Daily life management

1) Diet

During and after treatment, patients should maintain a rich and balanced diet that is safe and hygienic and good eating habits. Adequate intake of vitamins, minerals, proteins, carbohydrates, fats and water is important for treatment and rehabilitation. Specific dietary advice can be consulted with the nutritionist at your hospital.

2) Rest and exercise

Regular and quality sleep is very helpful for immunity and physical recovery, so patients need sufficient sleep time and good sleep quality. Providing a good sleep environment will help improve the patient's sleep quality, such as keeping the environment quiet, noise-free, dark light, temperature appropriate, etc.

If the patient's physical condition allows, they should be encouraged and assisted to engage in appropriate activities. Such activities can boost physical strength, stimulate appetite, and aid in treatment and recovery. Whether the patient is physically fit enough to participate in physical activities should be determined by a doctor. It is important to note that certain patients may need to limit their physical activity to prevent sports injuries, such as those with bone injuries or central nervous system issues leading to balance problems, ataxia, or difficulty walking.

3) Emotional psychology

The patient's emotional and psychological changes should be observed, and professional psychological intervention should be sought when necessary.

4. Daily disease monitoring

During the treatment, it is important to monitor for any side effects, complications, and the progression of the patient's condition. If any side effects or complications arise, consult a doctor immediately. After the treatment, continue to monitor for any aftereffects, the child's growth and development, and the likelihood of recurrence. Regular follow-up visits are essential. If new symptoms or aftereffects appear, consult a doctor promptly.

5. Special Precautions

All medical records of patients should be kept for reference during review and medical treatment.

6. Prevention

Because the exact cause of Langerhans cell histiocytosis is not clear, there is no corresponding prevention method.

Cutting-edge therapeutic and clinical Trials

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References

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2.Haupt R, Minkov M, Astigarraga I, et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical workup, and treatment for patients till the age of 18 years [J]Pediatr Blood Cancer, 2013, 60(2):175-184.

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4.Li D，H Li，Shi H． Clinical features and prognosis of Langerhans cell histiocytosis in children: an analysis of 34 cases. Chin J Contemp Pediatr，2017,19( 6) : 627-631.

5. National Health Commission of the People's Republic of China. Guidelines for the Diagnosis and Treatment of Rare Diseases (2019 edition). 2019.

6.https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq

7.https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq

8.Frade AP，Godinho MM，Batalha ABW，et al. Congenital Langerhans cell histiocytosis: a good prognosis disease? An Bras Dermatol，2017,92( 5) : 40-42.

9. Luo Danqing, Shi Lixiao, and Shi Xiaodong. Darafenib is effective in treating a case of Langerhans cell histiocytosis with BRAF-V600E gene-positive mutation, along with a literature review. Chinese Journal of Practical Pediatrics. 2017,32(17):1345-1347.