Langerhans Cell Histiocytosis


What is Langerhans Cell Histiocytosis?

The medical condition known as Langerhans Cell Histiocytosis is a rare disorder that may cause damage to other organs of the body. The symptoms experienced by patients depend on the organs that are affected [1, 2].

When a person has a Langerhans Cell Hisyiocytosis (LCH), there is an overproduction and proliferation of Langerhans’ cells. These cells are produced by the bone marrow and has a role in the inflammation response of the body.

They are named after the scientist Paul Langerhans who first discovered them in 1968. Cellular aberration can lead to uncontrolled growth and multiplication of these cells resulting to their accumulation in various parts of the body. Proliferation of LCH cells may result in organ damage or lesion formation [2, 3, 4, 5].

Langerhans Cell Histiocytosis


langerhans cell histiocytosis

Figure 1 – Langerhans Cell Histocytosis

This condition affects individuals from infants to adults although they more frequently occur in those who are between 0-15 years of age. The prognosis of LCH depends on the organ that is affected. It ranges from a self-healing bone lesion to a widely spread condition that is life-threatening.


Most of the cases have a good prognosis in which around 90% of those who undergo management are completely healed after finishing the entire course of treatment [1, 4].

Causes and Risk Factors

The exact mechanism that leads to the accumulation of Langerhans cells is still unknown but it is associated to immune regulation disorders. There are risk factors which may increase the possibility of developing LCH. Having a familial history of any type of cancer and thyroid disease may put the person is a risk factor.


Someone who is exposed to wood dust, granite, metal and benzene puts their offspring at higher risk for LCH. People who have acquired an infection while they were infants may develop LCH later in their life.  [1, 2].

Signs and Symptoms

The signs and symptoms experienced by patients is not the same because it depends on the organs that are affected by the accumulation of the cells. The development of lesion over the bone may cause different symptoms and the most common site for these bone lesions are the ribs, femur and the skull.

Joint pain is a common symptom among these patients and this pain is persistent during activity or even at rest. There may be swelling of the soft tissues over the bone. Although it rarely happens, these lesions may even lead to pathological fractures [1, 2, 4].

Proliferation of the Langerhans’ cells on the skin will manifest as skin rashes. These rashes may be petechial, papular, macular or nodular in nature. Lesions that will develop on the scalp will make it appear scaly. Ulceration of the mucosa and bleeding is also a common occurrence to these patients [1, 2, 4].

Involvement of the lungs will cause difficulty in breathing, nonproductive cough and spontaneous pneumothorax. The chest X-ray of these patients will yield abnormal results. Some of the rare symptoms it can cause include chest pain and hemoptysis [1, 2, 4].

Other symptoms that LCH can cause are enlargement of lymph nodes, hepatomegaly, splenomegaly, anorexia, night sweats, fever and weight loss [1, 2, 4].

Diagnosis

Health history and physical examination

The patient seeks consultation because of the presenting signs and symptoms. The physician will elicit a health history and familial history of the patient to aid in establishing the diagnosis. A physical examination will be performed to identify other symptoms that may be present. The physician may then ask for additional tests depending on the organs that are affected by LCH [1, 5].

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Imaging studies

Imaging tests such as X-rays, Magnetic Resonance Imaging (MRI) and Computed Tomography scan may be performed to identify the extent of the lesions. These tests will be able to tell the physician if the lesions are located in a single place or if several organs are already affected. The X-ray is also essential if the involvement of the lungs is being considered [1, 5].


Biopsy

The gold standard in the diagnosis of LCH is performing a biopsy. The physician will take a sample of the lesion and examine it under a microscope. The sample tissue may show histiocytes, eosinophils and Langerhans’ cells that are multinucleated [1, 5].

Treatment

The management for this condition depends on the number of the organs that are affected. There are several treatment modalities depending on the location. Bone lesions that are localized may be treated with intralesional steroids, chemotherapy and radiotherapy. If the lesion is accessible, a surgical curettage may be performed to remove the lesion [1].

Cutaneous manifestation of LCH are may resolve spontaneously without any treatment but if it persists topical nitrogen mustard and steroids may be used. Another option that can be chosen is the Psorlen with ultraviolet A (PUVA) therapy [1].

For patients who have pulmonary langerhans cell histiocytosis, the most part of the treatment is the cessation of cigarette smoking and this will improve the symptoms in some patients. Corticosteroids is the preferred treatment for this form of LCH.

If the lesion failed to respond to these drugs, the patient may need to undergo chemotherapy. Chemotherapeutic drugs are considered to be a last resort because of the risks associated with the use of these drugs. Transplant of the lungs may be required for advanced forms of pulmonary LCH [1].

If there is multi-organ involvement, the patient may be given vinblastine, prednisolone and 6-mercaptopurine. Patients who failed to respond to the treatment modalities previously done may need to undergo stem cell transplant [1].

Continuous monitoring is recommended to these patients because complications may occur many years after the primary lesions have resolved. It is estimated that about 50% of patients with LCH develop complications either due to LCH or to the treatment they underwent [1].

Pictures

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References
1. Hartree, N. (2014, January 23). Langerhans’ Cell Histiocytosis. Retrieved from Patient.info: http://patient.info/doctor/langerhans-cell-histiocytosis
2. National Cancer Institute. (2016, April 6). Langerhans Cell Histiocytosis Treatment (PDQ®)–Patient Version. Retrieved from National Cancer Institute: http://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq
3. Shea, C. R. (2016, February 8). Langerhans Cell Histiocytosis. Retrieved from Medscape: http://emedicine.medscape.com/article/1100579-overview?pa=MgaRCTAT28WAqEJTClW4MmYbqAoSjc0FdfpPYSnlNYDzzDqRJ5B%2FeA1ud3yw9hBU0Oa6T%2BYfpf4pNEhRwo1pnQkUAoK%2FTsPq9nb0yuBwgs0%3D#a6
4. Histiocytosis Association. (2015). LCH in Children. Retrieved from Histiocytosis Association: http://www.histio.org/lchinchildren#.V9JNh5h97IU
5. McClain, K. L. (n.d.). Langerhans Cell Histiocytosis. Retrieved from National Organization for Rare Disorders: http://rarediseases.org/rare-diseases/langerhans-cell-histiocytosis/

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