ЭВОЛЮЦИЯ ЛЕЧЕНИЯ ДЕТЕЙ С ГИСТИОЦИТОЗОМ ИЗ КЛЕТОК ЛАНГЕРГАНСА

Abstract

Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm caused by mutations in proteins in the intracellular signaling MAPK (mitogen-activated protein kinase) pathways. Its rarity, wide range of clinical manifestations, and varied course made it difficult to conduct clinical trials. The use of different stratification systems made it even more difficult to compare the published results. The review attempts to summarize approaches to treatment of LCH in children since the 1960s. Materials and methods of research: searches for original papers in PubMed were performed using combinations of the keywords Langerhans cell histiocytosis, X histiocytosis, pediatrics, children, treatment, and therapy. Only full articles published in English and German languages were included in the review. Conclusion: a combination of vinblastine and prednisolone as an intensive initial phase (6–12 weeks) followed by a less intensive maintenance phase of 12 months is the standard treatment of disseminated LCH. Mortality in multisystem LCH is caused by damage to the organs at risk (hematopoiesis, liver and spleen) and is especially high (up to 60–70%) if the patient does not respond to standard treatment. In the remaining patients, mortality is almost negligible, with the main problems being the recurrence of the disease and its irreversible effects in 50% of the survivors. Although systemic therapy has clearly improved survival in patients with the most severe disease, its role in sustaining disease control and prevention of irreversible consequences in patients with low-risk disease is less obvious.