Causes, manifestation and treatment of histiocytosis from Langerhans cells

Histiocytosis from Langerhans cells is a disease characterized by abnormal reproduction and accumulation of histiocytes( Langerhans cells) with diffuse or focal infiltration of organs and tissues( bone marrow, lungs, spleen, skin, lymph nodes, liver and other organs).

Frequency of occurrence and risk factors

Frequency of occurrence of Langerganscellular histiocytosis 1: 500000.Most often the children of the younger age are ill, the average age is 3 years. Children are much less likely to suffer from adolescence. There are cases of illness in adults, as a rule, these are elderly people. Men are sick much more often than women.

This disease is not hereditary, although it can sometimes be said about hereditary predisposition.

In adults, the lung form of Langerhans' histiocytosis is more common, smoking is the risk factor in this case.

Classification of

1. Number of organ systems affected:

• monosystem( single organ or multiple organ damage within one organ system)
• polysystemic( multiple organ systems)

2. Number of foci:

• single-focus( single lesion)
• polyocidal(several lesions)

3. In violation of the function of vital organs:

• with dysfunction of vital organs
• without dysfunction of vital organs

Causes and pathogenesis

Moretvuet two main theories regarding the aetiology of Langerhans histiocytosis.

1. Immunopathological. In favor of this theory is the fact that the frequency of spontaneous remission is high enough, relatively low mortality( in childhood - 15%, in adults - 3%), in the cells of the lesions there are no chromosomal abnormalities.
2. Tumor. In favor of this theory is the presence of a clonal type of proliferation of histiocytes in lesions.

In the pathogenesis of the disease, the main role is played by cytokines, which are produced by T-lymphocytes and histiocytes in the lesion site. Because of what the cells accumulate inflammatory infiltrate - macrophages, eosinophils, giant multinucleate cells. As a result, destruction and destruction of surrounding tissues occurs.

Clinical manifestations

Clinic of histiocytosis from the cells of Langerhans is very diverse and is caused by the fact that many organs and organ systems are affected. Consider the most frequent symptoms.

• Nonspecific symptoms: sleep disturbance, decreased appetite, fever, restlessness, most common in children of the first year of life.
• Bone lesions: the most common symptom, occurs in 80-90% of cases. The lesion focus can be single, it can be multiple. Osteolysis( destruction of the bone) is observed more often in the bones of the skull, femur, bones of the lower leg, and often the pelvis and ribs are affected. Other bones are less likely to be affected. Defects can be oval or irregular in shape. In their projection, there may be soreness and swelling. If the temporal bone is damaged, there may be a hearing impairment.
• Skin lesions are polymorphic, there may be a papular rash with a hemorrhagic component, or crusts and ulcers, there may be xanthomas, on the pilar part of the head there is seborrhea.
• Lesions of mucous membranes can be manifested by stomatitis, in girls - vulvovaginitis.
• If the foci are in the lungs, then probably the appearance of dyspnoea and an excruciating cough. In this case, regional lymph nodes are often affected.
• In liver and spleen lesions, there is an increase, in a delayed period, cirrhosis and hepatic insufficiency may occur. There may be jaundice and hyperbilirubinemia.
• When infiltrating the intestine can be diarrhea, hemocolitis, malabsorption.
• When the lesion spreads to the bone marrow, cytopenia appears.
• The pituitary gland is often affected, which causes hormonal disorders, in particular, diabetes insipidus.

Diagnostic Methods

1. Biopsies of lymph nodes, tissues and organs and their morphological investigation for the detection of Langerhans cells, when they are detected for more accurate diagnosis, immunocytochemical analysis and electron microscopy are used.
2. To assess the degree of organ damage, additional methods of examination are used: MRI, CT, lung and bone radiography, as well as a general blood test, a general urine test, a biochemical blood test.
3. With a single focus, corticosteroid therapy, local irradiation, surgical excision is used.
4. If the skin is damaged additionally - UV irradiation with photosensitizers.
5. In the common form - chemotherapy with the use of cytostatics.
6. When a bone marrow is affected, it is transplanted.

Treatment of Langerganscellular Histiocytosis

1. With a single focus, corticosteroid therapy, local irradiation, surgical excision is used.
2. If the skin is damaged additionally - UV irradiation with photosensitizers.
3. In the common form - chemotherapy with the use of cytostatics.
4. When a bone marrow is affected, it is transplanted.