Histiocytosis is a group of rarely diagnosed diseases of the hematopoietic system, which are classified as neoplastic and autoimmune diseases. Diseases in this group most often affect children from 1 to 6 years of age, although they can also occur in adults. What are the causes and symptoms of histiocytosis? What is the treatment of this type of disease?
Histiocytosisis a group of hematological diseases (hematopoietic system), the essence of which is the proliferation (uncontrolled proliferation) of histiocytes - cells of the immune system, which then accumulate in tissues and organs, gradually leading to their damage and failure.
Histiocytosis occurs by many names, including Hand-Schüller-Christian disease, multifocal eosinophilic granuloma, idiopathic chronic jaundice, LCH, and Abta-Letter-Siwegoto disease.
Currently, there is a localized form of the disease in which they are (bone / bones, skin and / or lymph nodes) and a diffuse form with multiple organ involvement, but both are very rarely diagnosed (2 cases per million) . Histiocytosis most often affects children from 1 to 6 years of age.
According to the World He alth Organization (WHO) classification, there are 3 classes of histiocyte proliferation:
1. Langerhans Cell Histiocytosis (LCH):
- eosinophilic granuloma
- Hand-Schuller-Christian disease
- Abt-Leterer-Gray disease
2. Hemophagocytic-lymphohistiocytic syndromes:
- Rosai-Dorfman disease - sinus histiocytosis with massive lymphadenopathy
- lymphohistiocytosis with haemophagocytosis
3. Acute monocytic leukemia:
- malignant histiocytosis
- histiocytic lymphoma
Histiocytosis: causes
The causes of the disease are unknown, but some researchers speculate that the growth of histiocytes is caused by overstimulation of the immune system, which leads to the accumulation of pathologically altered histiocytes in various organs.
Recently, more and more attention has been paid to the possible genetic basis of histiocytosis.
Histiocytosis: symptoms
Histiocytosis most often consists of:
- fever
- rashskin
- bone pains
- nodules in the scalp
- with a protruding eyeball
- enlarged lymph nodes
- enlarged liver
- enlarged spleen
Symptoms that may suggest histiocytosis also include sore gums, loosening of the teeth and premature loss, and in the youngest, a soft bulge in the bones of the cranial vault and long-lasting cradle cap.
Some patients experience retarded growth and sexual maturation as well as compression fractures of the vertebrae caused by lesions in the bones of the spine.
Histiocytosis: treatment
Treatment of histiocytosis depends on the form of the disease. A single focus can be removed (cured). Systemic chemotherapy is indicated in the case of multi-system disease and when individual disease foci cannot be cured by other methods.
Then the therapy usually includes cytotoxic drugs in combination with systemic steroids.