Causes, manifestations and treatment of histiocytosis of Langerhans cells

Histiocytosis from Langerhans cells - a disease characterized by the abnormal multiplication 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).

Incidence and risk factors

The incidence of Langerhans histiocytosis of 1: 500,000.Most often affects children younger, the average age - 3 years.Much less often sick children in their teens.There are cases in adults, usually older people.Men suffer more often women.

This disease is not hereditary, although sometimes one can speak of a genetic predisposition.

In adults, more common form of pulmonary Langerhans histiocytosis, a risk factor in this case is smoking.

Classification

1. According to the number of affected organ systems:

  • monosistemny (failure of one organ or several organs within the same organ system)
  • polisistemny (loss of several organ systems)


2.By the number of foci:

  • monoochagovy (one lesion)
  • poliochagovy (multiple lesions)

3. According to dysfunction of vital organs:

  • dysfunction of vital organs
  • without dysfunction of vital organs

Causes and pathogenesis

There are two main theories regarding the aetiology of Langerhans histiocytosis.

  1. immunopatologicheskih.This theory suggests that the spontaneous remission rate is high enough, the relatively low mortality (in childhood - 15% in adults - 3%) in the cells of the lesion no chromosomal abnormalities.
  2. Tumor.In favor of this theory is evidenced by the presence of clonal type proliferation of histiocytes in the lesions.

in the pathogenesis of the disease play a major role cytokines that are produced by T-lymphocytes and histiocytes in the lesion.Because of what in the outbreak accumulate inflammatory infiltrate cells - macrophages, eosinophils, multinucleated giant cells.As a result, and there is the defeat and destruction of the surrounding tissues.

Clinical manifestations

Clinic histiocytosis of Langerhans cells are very diverse and due to the fact that affects many organs and organ systems.Consider the most common symptoms.

  • nonspecific symptoms: sleep disturbance, loss of appetite, fever, restlessness, most common in infants.
  • bone lesions: The most common symptom, occurs in 80-90% of cases.The lesion may be a single, it can be multiple.Osteolysis (bone destruction) occurs more frequently in the bones of the skull, femur, tibia bones are also often affected pelvis and ribs.Other bones are affected less frequently.Defects can be oval or irregularly shaped.These projections may be soreness and swelling.With the defeat of the temporal bone can be hearing loss.
  • polymorphic skin lesions may be papular rash with hemorrhagic component or crusts and ulcers, can occur xanthomas on the scalp seborrhea occurs.
  • mucosal lesions may occur stomatitis, girls - vulvovaginal.
  • If lesions are found in the lungs, it is likely that the appearance of shortness of breath and painful cough.It often affects the regional lymph nodes.
  • If it affects the liver and spleen on the increase in the deferred period, there may be liver cirrhosis and liver failure.You may experience jaundice and hyperbilirubinemia.
  • When bowel infiltration may be diarrhea, gemokolit, malabsorption.
  • When distributing lesions on bone marrow cytopenia appears.
  • often affects the pituitary gland, which causes hormonal disturbances, in particular diabetes insipidus.

Diagnostic methods

  1. biopsy of lymph nodes, tissues and organs and their morphological study to identify the Langerhans cells, they identify for more accurate diagnosis using immunocytochemistry and electron microscopy.
  2. To assess the extent of organ damage using additional methods of examination: MRI, CT, X-ray of lungs and bones, as well as complete blood count, urinalysis, blood chemistry.
  3. When a single locus used corticosteroid therapy, local radiation, surgical excision.
  4. If it affects the skin further - UV irradiation with photosensitizers.
  5. When common form - chemotherapy with cytostatics.
  6. If it affects the bone marrow - transplant it.

Treatment Langerhans histiocytosis

  1. When a single locus used corticosteroid therapy, local radiation, surgical excision.
  2. If it affects the skin further - UV irradiation with photosensitizers.
  3. When common form - chemotherapy with cytostatics.
  4. If it affects the bone marrow - transplant it.
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