What is Histiocytosis X?
Histiocytosis X Disease, is a disease in which langerhans cells abnormally proliferate and can not function normally.
Normally, langerhans cells are cells that are responsible for protecting our body against viruses, bacteria and other invading microorganisms. Another name is Langerhans Cell In this disease, which is Histiocytosis, the langerhans cells are beginning to give damage to our bodies.
Is Histiocytosis X Disease Cancer?
The langerhans cells that cause cell and tissue destruction in our body are overgrown compared to the old ones. This view is similar to cancer.
In recent years studies have not accepted this disease as cancer.
Why is Histiocytosis X Disease?
The basic mechanism of the formation of this disease is still unknown. According to the United States statistics, one in every 250,000 children and one in every 1 million adults have this disease.
It is estimated that even a chest physician can see no more than 4 or 5 people caught in this disease throughout their career.
Who is seen?
In today ‘s conditions, the disease which can be diagnosed in the age range of 20 – 40 is very likely to be seen especially in smokers.
According to statistics, approximately 90 percent of patients with this disease are made up of smokers. Patients who present with symptoms such as shortness of breath and cough can be diagnosed incidentally after lung graphy is withdrawn.
What are Histiocytosis X Disease Symptoms and Findings?
We can list the signs and symptoms of Langerhans cell histiocytosis as follows:
- Swellings and aches in crows or stools
- Bone fractures that occur without trauma or due to minor injuries
- Jaw bones become loose in teeth after entrapment
- Hearing related problems after ear bones involvement
- Skin rashes on the scalp and in the calves
- Overdispersion in normal amounts in lymph glands
- Findings of jaundice and similar in deride and eyes
- Increased respiratory distress and cough complaints following lung involvement
- Hormone problems and excessive urination
Histiocytosis X is a difficult diagnosis because it is a very rare disease and it can give the symptoms given by other common diseases.
As your doctor may have bone, liver, lung or other organ involvement, your doctor will examine you in detail. Your doctor will be suspected on account of Computerized Tomography (CT), Positron Emission Tomography (PET) and other radiographs taken. However, because it is a very rare illness, your doctor needs to consider other diseases at the first stage and exclude them.
What is the definitive diagnosis of Histiocytosis X?
The definitive diagnosis will be made by biopsy after the suspicion of your doctor with the imaging methods mentioned above.
How is Histiocytosis X Disease Treatment done?
Treatment depends on the degree of progress of histiocytosis. First, it should be determined that the disease has only one organ involvement or multiple organ involvement. Our treatment plan has two varieties:
Treatment Plan for Single Organ Removal
When there is single organ involvement, prognosis is better than multiple organ involvement. Chemotherapy and corticosteroid treatments can be used in these patients. Only the form that holds the skin can be destroyed by chemotherapy applied to the skin. There are preventive treatments that reduce the severity of the disease according to the area of occupancy.
Treatment Plan for Multiple Organ Removal
Although the success rate of treatments is not as good as that obtained for single organ involvement, chemotherapy can be effective in these patients. It is known that many medicines have been tried for treatment, but the exact treatment is not yet known. Transplantation of organs such as the liver and lungs, which are often untreated, is another recommended treatment.