Histiocytosis corresponds to a group of diseases that can be characterized by the large production and presence of histiocytes circulating in the blood, which, although rare, is more frequent in men and its diagnosis is made in the first years of life, despite the indicative signs disease can also appear at any age.
Histiocytes are cells derived from monocytes, which are cells belonging to the immune system and are therefore responsible for the defense of the organism. After undergoing a differentiation and maturation process, the monocytes become known as macrophages, which are given specific names according to where they appear in the body, being called Langerhans cells when found in the epidermis.
Although histiocytosis is more related to respiratory changes, histiocytes can be accumulated in other organs, such as skin, bones, liver and nervous system, resulting in different symptoms according to the location of the greatest proliferation of histiocytes.
Main symptoms
Histiocytosis can be asymptomatic or progress to the onset of symptoms quickly. The signs and symptoms indicative of histiocytosis may vary according to the location where there is a greater presence of histiocytes. Thus, the main symptoms are:
- Cough; Fever; Weight loss without apparent cause; Difficulty breathing; Excessive tiredness; Anemia; Increased risk of infections; Clotting problems; Skin rashes; Abdominal pain; Convulsions; Puberty delay; Dizziness.
The large amount of histiocytes can result in the excessive production of cytokines, triggering the inflammatory process and stimulating the formation of tumors, in addition to causing damage to the organs where the accumulation of these cells is verified. It is more common for histiocytosis to affect bone, skin, liver and lungs, especially if there is a history of smoking. Less frequently, histiocytosis can involve the central nervous system, lymph nodes, gastrointestinal tract and thyroid.
Due to the fact that the children's immune system is poorly developed, it is possible that several organs may be affected more easily, which makes early diagnosis and the beginning of treatment immediately important.
How the diagnosis is made
The diagnosis of histiocytosis is made mainly by biopsy of the affected site, where it can be observed through laboratory analysis under a microscope, the presence of infiltrate with proliferation of histiocytes in the tissue that was previously healthy.
In addition, other tests to confirm the diagnosis, such as computed tomography, research of mutations associated with this disease, such as BRAF, for example, in addition to immunohistochemical tests and blood count, in which there may be changes in the amount of neutrophils, lymphocytes and eosinophils.
How to treat
The treatment of histiocytosis depends on the extent of the disease and the affected site, and chemotherapy, radiation therapy, use of immunosuppressive drugs or surgery, especially in the case of bone involvement, is recommended. When histiocytosis is due to smoking, for example, smoking cessation is recommended, significantly improving the patient's condition.
Most of the time, the disease can heal on its own or disappear due to treatment, however it can also reappear. For this reason, it is important that the person is monitored regularly so that the doctor can observe if there is a risk of developing the disease and, thus, establish the treatment in the early stages.
