Histocytosis is a group of diseases that can be characterized by the high production and presence of circulating histiocytes in the blood, which, although rare, is more common in humans and is diagnosed in the early years of life, despite the indicative signs of the disease can also occur at any age.
Histiocytes are cells derived from monocytes, which are cells belonging to the immune system and therefore responsible for the defense of the organism; after a process of differentiation and maturation, the monocytes are renamed macrophages, which receive specific names depending on where they appear. called Langerhans cells when they’re in the epidermis.
- Although histiocytosis is more related to respiratory changes.
- Histiocytes can accumulate in other organs.
- Such as the skin.
- Bones.
- Liver.
- And nervous system.
- Resulting in different symptoms depending on the location of the increased proliferation of histiocytes.
Histocytosis can be asymptomatic or progress rapidly toward the onset of symptoms; signs and symptoms of histocytosis may vary depending on where histiocytes are most present, so the main symptoms are:
The large number of histiocytes can lead to excessive cytokine production, triggering the inflammatory process and stimulating the formation of tumors, as well as causing damage to the organs where the accumulation of these cells is verified, being more common for histicytosis to affect the bones, skin, liver and lungs, especially if there is a history of smoking. Less often, histocytosis can affect the central nervous system, lymph nodes, gastrointestinal tract, and thyroid.
Due to the lack of development of children’s immune systems, several organs may be more easily affected, making early diagnosis and treatment immediately important.
The diagnosis of histicytosis is mainly done by biopsy of the affected site, where the presence of an infiltrate with histiocyte proliferation in previously healthy tissue can be observed by laboratory analysis under the microscope.
In addition, other tests to confirm the diagnosis, such as CT, the search for mutations associated with this disease, such as BRAF, for example, in addition to immunohistochemistry and blood formula tests, in which there may be changes in the number of neutrophils, lymphocytes and eosinophils.
Treatment of histhytocytosis depends on the extent of the disease and the affected site, and chemotherapy, radiation therapy, the use of immunosuppressive drugs or surgery are recommended, especially in case of bone damage. When histocytosis is caused by smoking, for example, quitting smoking is recommended, greatly improving the patient’s condition.
Most of the time, the disease can heal on its own or go away with treatment, but it can also recur, so it is important for the person to be monitored regularly so that the doctor can see if there is a risk of developing the disease and establish treatment at an early stage.