Histiocytosis of Langerhans: causes and treatment

Langerhans cells are white blood cells that are part of the immune system and under normal conditions play an important role in protecting the body from viruses, bacteria and other harmful microorganisms. They are found in the skin, lymph nodes, spleen, bone marrow and lungs.

What is Langerhans' histiocytosis?

In this disease, formerly known as histiocytosis X, Langerhans cells begin to multiply in pathological scales. Instead of protecting the body, large clusters of these cells disrupt the integrity of tissues and even destroy them. Bones, lungs and liver most often suffer. Although similar growth of cells has much in common with oncological diseases, most researchers do not consider histiocytosis X as a form of cancer. The main differentiating feature in this context is the normal structure of aggressive cells, and the only problem is their accelerated growth. Currently, the histiocytosis of Langerhans (in children, it should be noted, it is more common than in adults) is recognized as a disease of the immune system, in which immune cells multiply excessively rapidly and cause inflammation and damage to nearby tissues.

This disease can develop in any part of the body and in any organ. It can even appear in several different areas simultaneously. In most cases, however, pathology affects several body systems only in children under the age of two. Single foci are found in both children and adults.

Causes

It is still unknown why such a pathology arises. However, scientists are focused on testing the following hypothesis: most likely, some kind of common substance in the environment, possibly a viral infection, gives impetus to the development of the disorder, causing an over-intensive reaction from the immune system. However, the source of the disease itself is currently being identified. One well-known observation is known: almost all patients with localization of pathology in the lungs are active or former smokers of cigarettes. Nevertheless, even with the massive spread of smoking in society, it is very rare that Langerhans' histiocytosis occurs. Reasons to include the use of cigarettes in the list of risk factors as if there is not, because often the ailment does not affect the lungs at all. The most likely assumption is that the negative effect of smoking is realized by the proliferation of Langerhans cells in individuals with a rare genetic predisposition to it. According to another theory, the root cause of the disease lies in the congenital disorders of the immune system.

Spread

The disease under consideration is rare and occurs in approximately one case for 250,000 children and in one case for one million adults. Although the symptoms can occur at any age, it is most often diagnosed in children and adolescents, with 70% of cases of histiocytosis occurring at the age of 17 years. In the younger age group of patients, statistical indicators increase at the age from one to three years.

Forms of the disease

Histiocytosis from Langerhans cells in children and adults includes 3 forms previously considered independent pathologies. These are eosinophilic granuloma, Hend-Schuiller-Crischen disease and Letterter-Sieve disease.

Symptoms

The disease can be manifested by the following symptoms:

• Permanent pain and swelling on the bones, especially on the arm or leg;
• A crack in the bone, especially if it was not preceded by an injury or was preceded by a slight, minor stroke;
• Large gaps between teeth in cases where Langerhans' histiocytosis in children has spread to the jawbone;
• Ear infection or discharge from the ear if the disease touched the bones of the skull next to the ear;
• Skin rash with predominant localization on the buttocks or scalp;
• Enlarged lymph nodes;
• Enlargement of the liver, which in some cases may be accompanied by signs of organ dysfunction (yellowing of the skin and eyes (jaundice) or pathological accumulation of fluid in the abdominal cavity - ascites);
• Exophthalmos - forward bias of the eyeball caused by histiocytosis with localization behind the eyes;
• Difficulty breathing and coughing with lung damage;
• Slow growth and profuse urination due to hormonal disorders.

Less common symptoms of ailment are:

• Increased body temperature;
• weight loss;
• irritability;
• Inability to maintain weight, and also to support the body in the form.

Diagnostics

Since the histiocytosis of Langerhans (photos of the manifestations of this disease is not very pleasant sight) is quite rare and is expressed by such symptoms that it is easy to take as signs of many other diseases, diagnosis is often complex and time consuming. That is why the doctor will most likely ask you questions related to other diseases in order to more effectively differentiate the alleged histiocytosis.

The primary diagnostic examination is a routine medical examination, in which the doctor assesses the general condition of the body and pays special attention to those areas where the symptoms of pathology are manifested. If a patient experiences pain in the bone or complains of swelling on the bone, the doctor may prescribe a standard radiography of the bones and scintigraphy. X-rays will identify a site of destruction, called a lytic lesion, and scintigraphy will identify areas of damage where the bone tries to recover independently.

The method of computed tomography (CT) is used when examining the back and pelvis in cases when the doctor suspects that it is there that the centers of destructive changes may be located. With symptomatic signs of liver dysfunction, you will need to take blood tests. If signs of a lung injury or in cases when a specialist on the basis of a primary medical examination suspects that Langerhans' histiocytosis may be found in the lungs, an x-ray or CT scan of the chest is prescribed . CT of the head is performed to assess the state of the brain. A new imaging technology, PET (positron emission tomography), can also be useful.

If there is a suspicion of Langerhans' histiocytosis in children (see photo at the beginning of the article), a biopsy may be required, since this is the only way to confirm the alleged diagnosis. In this procedure, the doctor removes a small sample of tissue or bone for later study in the laboratory. In most cases, the sample of the biomaterial is extracted from the sites where the histiocytosis of Langerhans is directly localized: bones, skin and lymph nodes. Blood tests can help determine if the bone marrow is affected.

Duration

It is difficult to predict how fast the course of the disease will be and how it will manifest itself. In some cases, the pathology disappears by itself and does not require medical intervention. This occurs when the disorder is localized in one area (in bone or skin) or when a smoker with affected lungs stops consuming cigarettes. However, it also happens that the disease is fatal; A lethal outcome can be predicted in those cases where clusters of pathologically proliferating cells attack simultaneously several different organs or parts of the body. The health status of smokers begins to improve only if you quit smoking.

Prevention

Since the cause of the development of the violation remains a mystery, at the moment there are no effective preventive measures. However, given that the lungs are affected only by fans who smoke cigarettes, the rejection of addiction can be considered as a prevention of histiocytosis with localization in the lungs.

Treatment

If a patient has a confirmed diagnosis of "histiocytosis from Langerhans cells," the treatment will depend on the severity and extent of the disorder.

If the pathology is found only in one body system, the doctor will prescribe the use of corticosteroids, in particular "Prednisolone". Additionally, you may have to undergo chemotherapy. In addition, the affected area of the bone is removed using the curettage method - scraping. If pathologically proliferating cells are concentrated in one area of the skin, the disease can be cured by covering the affected area with nitrogen mustard, a chemotherapy agent. "Methotrexate" is also used. Radiotherapy is prescribed in cases where bone damage (for example, in the upper part of the leg or in the spine) has weakened the whole fragment of the skeleton. Bone-plastic surgery on the spine - spondylodesis - is recommended for instability of bones in the cervical region.

The generalized (systemic) histiocytosis of Langerhans is a much heavier condition. Chemotherapy can also be used for treatment, but its efficacy is significantly reduced compared to therapy for single lesions. Although experts have checked the effectiveness of many drugs, optimal medication has not yet been found. Nevertheless, in order to monitor the development of the disease, a doctor can prescribe one of the following:

• "Vinblastine";
• "Etoposide";
• "Mercaptopurine";
• "Cladribine";
• "Cytarabine";
• "Methotrexate."

In some cases, doctors recommend liver, bone marrow or lung transplantation, but among the researchers consensus on the optimality of organ transplantation in patients with histiocytosis has not yet been achieved.

When to see a doctor?

Be sure to contact your local pediatrician if you suspect Langerhans' histiocytosis in children. Ocular manifestations, pain or swelling on the bone, chronic skin rash are signs of this rare disease. In the presence of these or symptoms of pathology in adults, you should consult a therapist.