Гистиоцитоз (Histiocytosis)

Histiocytosis is a group of diseases caused by abnormal changes in macrophages (histiocytes). As a result, a person may develop various biochemical disorders.

One of the manifestations of histiocytosis is Gaucher disease. With this disease, abnormal deposition of fats occurs in the body due to metabolic disorders.

Other rare forms of histiocytosis include Hand-Schüller-Christian disease (a variant of reticuloendotheliosis) and Letterer-Sieve disease. These diseases are not fully understood, but are also associated with pathological changes in macrophages.

Histiocytosis can affect various organs and tissues. Clinical manifestations depend on the location and extent of the lesion. Diagnosis is based on the analysis of bone marrow cells and histological examination of biopsy specimens. Treatment includes chemotherapy, bone marrow transplantation and other methods depending on the form and severity of the disease.



Histiocytosis is a group of rare diseases caused by the accumulation and abnormal activation of histiocytes (a type of immune cell). Histiocytosis can affect a variety of organs and tissues, including bones, skin, lymph nodes, lungs, liver, spleen, and bone marrow.

There are three main types of histiocytosis:

  1. Langerhans cell histiocytosis is characterized by the accumulation of abnormal Langerhans cells in the body. Most often it affects the bones, lungs and skin.

  2. Hand-Schüller-Christian disease is a rare disease in which histiocytes accumulate in the bones, lungs, liver, spleen and lymph nodes.

  3. Malignant histiocytosis is an aggressive form that can affect many organs and is characterized by rapid, uncontrolled growth of abnormal histiocytes.

The causes of histiocytosis are not fully understood. It is believed that it may be associated with mutations in genes that control the growth and division of histiocytes. Diagnosis is based on analysis of clinical manifestations, laboratory tests and biopsy of affected tissue. Treatment depends on the type and extent of the disease. May include chemotherapy, radiation therapy, surgery, and bone marrow transplant. The prognosis also varies depending on the form of histiocytosis.



Histiocytosis is a rare disease that is caused by abnormal changes in immune system cells known as macrophages or histiocytes. These changes lead to the accumulation and abnormal functioning of histiocytes in various organs and tissues of the body. As a result, various biochemical disturbances and symptoms may occur.

One form of histiocytosis is Gaucher disease. With this disease, fatty substances accumulate in various organs, such as the liver, spleen and bones. This causes various problems, including increased organ size, impaired function, and possible tissue damage.

There are other forms of histiocytosis, such as Hand-Schüller-Christian disease and Letgerer-Siwe disease. Hand-Schüller-Christian disease is characterized by damage to the nervous system and can lead to a variety of neurological symptoms, including psychomotor retardation, seizures and vision loss. Letgerer-Sieve disease is caused by a deficiency of a specific enzyme and also affects various body systems, including the skin, intestines and liver.

Histiocytosis is a complex disease and its exact causes are not fully known. However, there are various hypotheses related to disorders in the immune system or genetic mutations that may play a role in the development of this disease.

The diagnosis of histiocytosis is based on clinical symptoms, laboratory results, and examinations such as biopsies of affected tissue. Treatment for histiocytosis depends on its type and severity. In some cases, chemotherapy, drugs, or surgery may be necessary.

It is important to note that histiocytosis is a rare disease, and each specific case requires an individual approach to diagnosis and treatment. Consultation with experienced medical specialists such as oncologists or hematologists is an important part of managing this condition.

In conclusion, histiocytosis is a group of rare diseases that cause abnormal changes in the cells of the immune system. This can lead to various biochemical disorders and symptoms in various organs of the body. Gaucher disease, Hand-Schüller-Christian disease, and Letgerer-Siwe disease are some of the forms of histiocytosis. The diagnosis and treatment of histiocytosis require an individual approach and consultation with specialists. Early diagnosis and timely treatment can help manage this rare disease and improve the patient's quality of life.