Histiocytosis of Langerhans cells (class 1 histiocytosis) includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, HandSchllerChristian syndrome, and LettererSiwe diseases. This syndrome represents a spectrum of severity and prognosis of some underlying disorder ...
Langerhans cell histiocytosis (LCH) is a neoplastic disorder that arises from an expansion of early myeloid cells in the bone marrow compartment, most commonly due to a clonal mutation in the RAS/MAP Kinase signaling pathway. This process results in the dissemination of CD1a and CD207 (Langerin...
Clinical Classification of Langerhans Cell Histiocytosis (LCH)1 TypeClinical Features Unifocal Single site of involvement, most commonly bone; Older children and adults; Good prognosis Multifocal single system Multiple sites of involvement, most commonly bone; Young children; Intermediate prognosis ...
Langerhans cell sarcoma, or LCS, is a rare malignant tumor that affects Langerhans cells. It is a extremely rare tumor that has a poor prognosis... Learn more about this topic: Langerhans Cell Histiocytosis: Prognosis, Treatment & Symptoms from...
Systemic treatment incorporating steroids and cytostatic drugs for at least one year has improved prognosis of multisystem LCH and represents the current standard of care. 展开 关键词: BRAF Langerhans cell histiocytosis MAPK pathway myeloid neoplasia ...
Langerhans cell histiocytosisorbitThe clinical manifestations of Langerhans cell histiocytosis have been recognized for more than a century. For most of that time, physicians have viewed the disease from different perspectives, interpreting portions of its clinical spectrum as if they were distinct and ...
Optimal therapy for Langerhans cell histiocytosis (LCH), particularly in the case of multisystem organ disease, is emerging. The Histiocyte Society has conducted a number of prospective, randomized control trials to study the effect of various chemotherapeutic regimens in the treatment of LCH, which ...
Histiocytoses are rare disorders of unknown origin with highly heterogeneous prognosis. BRAFV600E gain-of-function mutations have been observed in 57% of cases of Langerhans cell histiocytosis (LCH) and 54% of cases of Erdheim-Chester di... Drier,Aurelie,Hervier,... - 《Blood》 被引量: 43...
3.Krooks J, Minkov M, Weatherall AG. Langerhans cell histiocytosis in children: History, classification, pathobiology, clinical manifestations, and prognosis.J Am Acad DermatolDOI[Cited in This Article:3][Cited by in Crossref: 81][Cited by in F6Publishing: 120Reference Citation Analysis (0)] ...
The natural history of Langerhans cell histiocytosis varied from an acute fulminant course, a waxing and waning chronic disease, to spontaneous regression. Young age at presentation and organ dysfunction predicted a poor prognosis. Statistical analysis showed that there was no significant relationship ...