Letterer-Siwe Disease

Letterer-Siwe Disease is a rare disease belonging to the group of reticuloendotheliosis. This is a severe form of histiocytosis, mainly affecting children under 3 years of age.

The disease is characterized by the proliferation and accumulation of abnormal histiocytes (cells of the reticuloendothelial system) in various organs and tissues. The bone marrow, liver, spleen and lymph nodes are most commonly affected.

KLIN Severe generalized proliferation of histiocytes leads to dysfunction of the affected organs. Hepatosplenomegaly, anemia, thrombocytopenia, and immunodeficiency are noted. Systemic manifestations are typical - fever, weight loss, diarrhea.

Letterer-Sieve disease usually progresses rapidly and is fatal. The average life expectancy after diagnosis is about 1 year. Treatment is mainly symptomatic and supportive. Chemotherapy may be used. The prognosis is unfavorable, mortality approaches 100%.

Letterer Sieve's disease (LSD) is a rare chronic disease from the group of hypereosinophilic syndromes, characterized by the formation of multiple affected lymph nodes and involvement of other organs. This rare cancer is often associated with severe eosinophilia in the peripheral blood and elevated levels of eosinophils in the bone marrow, which distinguishes it from other forms of hypereosinophilic syndromes. The disease occurs in different geographic regions, although it is more common in the Western world than in Japan and some developing countries. It is also most commonly seen in children and young adults.

Causes of Letterer's disease

The cause of the disease is the accumulation of a large population of abnormal T-helper-2 lymphocytes, caused by mutations in the _STAT3_ gene on chromosome 22q11.2. This leads to the expression of IL-13 and other chemokines. Induction of inflammation in the lymphoid system can provoke the onset and development of peripheral lymph nodes, as well as damage to the liver, spleen and central nervous system. Symptoms can vary greatly between patients and depend on the location and severity of the lesion. In half of the patients it is only leukoplakia (lesions in the form of white spots), which occurs in the area of ​​inflammation and can be found in the liver or upper respiratory tract. The other half have developing lymphadenopathy, diffuse lesions of the skin, abdomen, liver and neurological symptoms. These changes usually develop gradually over several years, but can be triggered by infections