Histiocytosis 101
Histiocytosis is defined as pathological proliferation of white blood cells. The most common histiocytosis is Langerhans cell histiocytosis.
What is histiocytosis?
According to abbreviationfinder, Histiocytosis is a rare, different form of disease in which there is a pathological proliferation of special white blood cells called histiocytes . Most patients suffer from Langerhans cell histiocytosis (LCH), also known as histiocytosis X.
Other forms are Erdheim-Chester disease, juvenile xantogranuloma and Rosai-Dorfman disease. While juvenile xantogranuloma and Rosai-Dorfman disease are usually benign, Erdheim-Chester disease affects multiple organs and the skeleton.
Even the brain can be affected. Therefore, this form of histiocytosis is considered life-threatening. As already mentioned, Langerhans cell histiocytosis is the most common form of histiocytosis. In Germany, around 40 to 50 children develop this form of histiocytosis every year, which is also known as Abt-Letter-Siwe syndrome, Hand-Schüller Christian syndrome or eosinophilic granuloma.
Between 70 and 80 percent of the sick children are younger than ten years. Histiocytosis is much less common in adult humans, although it is assumed that its frequency is underestimated.
Causes
Histiocytosis is a disease of the monocyte/macrophage system. These are body cells that form in the bone marrow and are found in all organs. They are important to ward off foreign substances. The various histiocytosis have in common that the histiocytes multiply pathologically in one or different organs.
The causes of histiocytosis are unknown. Langerhans cell histiocytosis is not inherited and is not contagious. It almost always shows itself in childhood. However, cases of the disease in adults have increased in recent years. Langerhans cell histiocytosis can be divided into three subtypes.
These are Abt-Letter-Liwe syndrome, which usually occurs in babies and young children, Hand-Schüller-Christian syndrome, which occurs primarily in young children, and eosinophilic granuloma, which arises on the bones and develops between the ages of 5 and 5.and 20 years of age.
Symptoms, Ailments & Signs
The symptoms that occur with histiocytosis are manifold. However, there are quite similar complaints, which can give clues to the disease. The most common general symptoms include pain in the affected body parts, exhaustion, fever, visual disturbances such as squinting or bulging eyes, increased incidence of infections, coughing, breathing problems, severe thirst and chronic middle ear infections.
In addition, patients suffer from swelling, pale skin, diarrhea, loss of urine, growth disorders, swollen gums and loose teeth, which often fall out. In addition, there are rashes on the skin that resemble diaper rash. Langerhans cell histiocytosis is generally considered to be a benign disease.
In some cases, however, its course can be so severe that it assumes life-threatening proportions. Isolated involvement of the lungs or individual bones is often found in adult patients. However, multifocal disease outbreaks are also possible with them.
Diagnosis & disease progression
Due to the numerous different symptoms, it is not always easy to diagnose histiocytosis. Misdiagnoses are not uncommon, especially in the initial phase. However, if the suspicion of the disease is confirmed, a tissue removal ( biopsy ) takes place. During the analysis, care must be taken to clearly differentiate between Langerhans cell histiocytosis and the other forms.
X-rays and computed tomography (CT) can be used as further examination methods. X-ray examination can be used to determine the breakdown of bones, while computed tomography can be used to diagnose lung involvement. An important indication for the diagnosis of histiocytosis X is the detection of a flat vertebra.
In most patients, Langerhans cell histiocytosis takes a positive course. If the disease becomes chronic, it can lead to problems in the spine or loss of teeth. Some babies do not respond to treatment, which can lead to the disease becoming life-threatening.
Ultimately, no definitive statement can be made about a positive or negative course at the start of therapy, so that each child is an individual case. Basically, the cure rate is around 70 percent.
Complications
As a result of histiocytosis, the affected person suffers from various complaints that drastically reduce the quality of life and significantly restrict the patient’s everyday life. As a rule, the patient has reduced resilience and severe exhaustion. Furthermore, visual disturbances and hearing problems occur, whereby in the worst case the affected person can go completely blind. Middle ear infections are not uncommon.
Due to the weakened immune system, various diseases and infections occur more frequently. In children, growth disorders and further delayed development of the body can occur. The skin is usually covered with rashes and is relatively pale. In many cases, the histiocytosis also leads to mental disorders or depression.
The treatment of histiocytosis usually always depends on the affected region. Surgical interventions or various creams and ointments can be used to eliminate the symptoms of histiocytosis. Furthermore, the patient must give up smoking, for example, in order for the disease to progress positively. In some cases life expectancy is limited by histiocytosis.
When should you go to the doctor?
If symptoms such as fever, exhaustion and visual disturbances are noticed, often associated with non-specific pain, histiocytosis may be the underlying cause. A doctor’s visit is recommended if other typical symptoms such as breathing problems or severe thirst appear. If the symptoms increase in intensity and do not go away on their own, a doctor must be consulted. In any case, histiocytosis requires medical treatment. Therefore, those affected with persistent symptoms should consult a doctor.
Medical advice is required at the latest when swelling, noticeably pale skin and swollen gums are noticed. Since babies and young children are mostly affected, parents are advised to watch out for abnormalities, especially if other family members have the disease. In the event of serious complications such as infections or cardiovascular problems, it is best to call the emergency services. In case of doubt, the medical emergency service can be contacted first. The right contact person for histiocytosis is the general practitioner or an angiologist. The dentist can be consulted for symptoms of teeth and gums.
Treatment & Therapy
The therapy of histiocytosis depends on the subform that occurs in each case. For example, if the disease is limited to a single section of bone, there is the option of surgically removing the focus. If the section is in a part of the body that is not suitable for surgery, such as a joint, radiation is an alternative.
If the histiocytosis occurs in several parts of the body, mild chemotherapy is carried out. If lymph nodes are affected, they can be surgically removed. Langerhans cell histiocytosis can also resolve on its own in some patients without specific treatment. If the skin is affected, however, the administration of cortisone is considered effective.
For this purpose, creams or ointments containing the active ingredient are applied to the affected areas of the skin. Adult patients with isolated lung disease must consistently stop smoking. Study results showed that the nicotine contained in tobacco products triggers the lung attack.
Unless the course of the disease is severe, no further therapeutic measures are necessary. However, there are still different opinions among medical professionals. While children suffering from histiocytosis are treated in a university hospital, adult patients have to see different specialists.
Outlook & Forecast
Children have the best chance of curing histiocytosis. Under favorable conditions, freedom from symptoms is often achieved in them. With a restructuring of living conditions and treatment, recovery can be documented in the majority of patients. Nevertheless, they can also develop a chronic course of the disease. This applies in particular to early onset of the disease. Problems in the spine or a loss of teeth are treated in various therapeutic approaches, but cannot be regenerated in the way they work under natural conditions. In severe cases, the disease can become life-threatening. If the initiated therapy is unsuccessful, there is a risk of a steady increase in health impairments,
In adults, the overall prognosis is more favorable. Only rarely does life endanger or a shortening of the expected lifespan. If the doctor’s instructions are followed and smoking is incompletely avoided, a significant reduction in symptoms can be achieved with extensive treatment. Some patients are already experiencing a significant improvement by changing their lifestyle.
If the disease is in the early stages, the patient often does not need to undergo any medical treatment. A natural regeneration of the symptoms takes place, so that the patient is then discharged from the treatment as cured.
Prevention
Preventing histiocytosis is not possible because it is inherited. In addition, their triggering causes are not known.
Aftercare
As a rule, the affected person has either very few or even no measures and options available for aftercare. In any case, those affected are dependent on early and, above all, rapid diagnosis and treatment so that further complications or symptoms can be prevented. Self-healing is also not possible, so that treatment always has to be carried out.
Early diagnosis with rapid treatment always has a very positive effect on the further course of this disease. In many cases, the histiocytosis is treated by surgical intervention. After such an operation, the person concerned should definitely take it easy and rest. Exertion or other physical and stressful activities should be avoided.
In many cases, a healthy lifestyle with a healthy diet also has a positive effect on the course of the disease. The affected person should also refrain from smoking and drinking alcohol if possible. Regular examinations of the body are also necessary in order to detect further damage to the body at an early stage. It is not uncommon for the life expectancy of those affected to be reduced by histiocytosis.
You can do that yourself
Histiocytosis is always treated depending on the respective subform. The measures that those affected can take themselves depend on whether only a single bone section is affected by the disease or several parts of the body.
Langerhans cell histiocytosis sometimes resolves on its own. The most important measure is then to have the blood values monitored by a doctor in order to be able to react quickly in the event of a recurrence. Widespread disease requires chemotherapy, which can be supported by a healthy lifestyle. Further exposure to nicotine or alcohol should be avoided, simply because of the risk of interactions with the cytostatics used. If the lungs are affected, giving up cigarettes is particularly important. Furthermore, rest and relaxation apply during and after chemotherapy, although sport is permitted in moderation and with the doctor’s consent.
Those affected can also take action themselves against the individual symptoms of histiocytosis. Rashes and eczema can be relieved with increased hygiene and the use of appropriate ointments. Drinking a lot helps against severe thirst. Breathing problems and coughing attacks can be reduced by inhaling a salt water solution. Which measures are particularly useful is best answered by the responsible doctor.
