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Titlebook: Histiocytic Disorders; Oussama Abla,Gritta Janka Book 2018 Springer International Publishing AG 2018 langerhans cell Histiocytosis.hemopha

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Hematopoietic Cell Transplantation and Novel Therapies in Hemophagocytic Lymphohistiocytosis HLH, HCT is essential for preventing future episodes of fatal HLH in patients with genetic predisposition to HLH. Survival with HCT has increased significantly in recent years with the adoption of reduced intensity conditioning (RIC) regimens. However, RIC treatment protocols carry their own distin
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Biology and Genomics of LCH and Related Disordersnslocations that produce fusion proteins which activate . occur more frequently in ECD than in LCH. The presence of some of these activating mutations in circulating cells or in CD34+ bone marrow cells suggests that these diseases may arise from transforming events in early myeloid precursors. ICH c
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Central Nervous System Langerhans Cell Histiocytosisnation. Biopsies of such lesions are rare. In the few published cases biopsied, variable pathologies are described with perivascular inflammatory changes, variable neuronal loss, demyelination, and gliosis, but are nondiagnostic for CD1a+/CD207+ LCH cells. Granulomatous LCH can be responsive to chem
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First-Line Treatment of Pediatric Langerhans Cell Histiocytosisactivation rates. The effect of further prolongation is being studied in the current LCH-IV protocol of the Histiocyte Society. It is to be expected that in the (near) future, the use of BRAF and MEK inhibitors may be introduced in first-line therapy, especially for very high-risk patients. For thes
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