Langerhans cell histiocytosis as a clonal disease of mononuclear phagocyte system
نویسندگان
چکیده
Langerhans cell histiocytosis (LCH) belongs to histiocytic proliferative diseases, which are rare in clinical practice; however they pose significant challenges both for their diagnosis and choice of therapeutic strategies. Histiocytic diseases the scope oncology; nevertheless, at diagnostic stage patients referred pediatricians or dermatologists. That is why interdisciplinary interaction various specialties common approaches classification, treatment important management with disorders.
 Accumulation studies on LCH pathophysiology has promoted development new algorithms methods. After fact MAPK signal pathway activation had been established, potential target therapy was identified. Neoplastic nature hypothesized. If confirmed, we can expect actual being elaborated, particular, predict disease depending tumor clone mutation type. The unique characteristics including proliferate clonality (presumable neoplastic nature), course spontaneous regression frequent relapses tropism certain tissues (target organs) make up grounds further in-depth disease.
منابع مشابه
Langerhans cell histiocytosis
Introduction: Langerhans cell histiocytosis (LCH), which was previously known as histiocytosis X, refers to a group of lesions that stimulate uncontrolled proliferation of cells. The purpose of this case was to report and describe a LCH case with mandibular bone involvement in a 2.5-year-old boy. Case reports: A 2.5 years old boy with LCH of mandibular jaw who has been treated by surgical inter...
متن کاملCurrent Treatment Strategy in Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH) is a rare disorder described as three different entities including eosinophilic granulomaof bone, the Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. LCH is currently classified into singlesystem LCH, and multisystem LCH. Patients with single system LCH have an excellent prognosis, and are mostlytreated with local therapy. Multisystem LCH is subd...
متن کاملLangerhans Cell Histiocytosis Followed by Hodgkin Lymphoma: A Case Report
Langerhans cell histiocytosis (LCH) is a rare neoplasm defined as the proliferation of bone marrow langerhans cells, which is a kind of dendritic cells. The major pathological features of LCH are expression of CD1a and S100 as well as Birbeck granules. Its presentation can differ from a mild bone lesion to a multi-systemic evolved malignant neoplasm; however, the latter outcome is almost rare. ...
متن کاملCentral nervous system disease in Langerhans cell histiocytosis.
Central nervous system (CNS) disease in Langerhans cell histiocytosis (LCH) is a poorly understood complication of yet unknown frequency. By far the most common manifestation is in the hypothalamic-pituitary system with diabetes insipidus as the leading sign, followed by other endocrinopathies and hypothalamic dysfunction. However, essentially all other parts of the CNS may be involved. On the ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Al?manah Klini?eskoj Mediciny
سال: 2023
ISSN: ['2587-9294', '2072-0505']
DOI: https://doi.org/10.18786/2072-0505-2022-50-057