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

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Treatment of Relapsed and Refractory Langerhans Cell Histiocytosis in Children their disease within 5 years, and the treatment of these patients has been a challenge and their outcome is very poor. Treatment failures in patients with low-risk LCH are not associated with mortality but with morbidity, and their optimal treatment is not well defined. Nucleoside analogues such as

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Adult Langerhans Cell Histiocytosisdult LCH patients are often evaluated by physicians who focus only on the most obviously affected organ without sufficient evaluation of other systems, which may lead to an erroneous diagnosis in some patients. In addition, treatment with pediatric-based regimens, often referred to as standard thera

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Late Effects of Langerhans Cell Histiocytosis and the Association of LCH with Malignancyy be already evident at the moment of LCH diagnosis or become manifest even more than 10 years after the end of therapy. They may be due either to the site of the original disease or to age at treatment and type and doses of treatments received..Since the bony skeleton is the most common site of inv

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Classification, Clinical Manifestations, and Diagnostics of HLHecondary form that complicates diverse infections, autoimmune or autoinflammatory disorders, and malignancies. Both forms, primary and secondary HLH, present with the same spectrum of non-specific symptoms, making accurate diagnosis and rapid treatment initiation challenging. A prompt diagnosis is c

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CNS Involvement in HLH (CNS-HLH)me in the further course of systemic HLH but has also been reported as primary or only manifestation. Diagnosis is based on the presence of neurological symptoms and abnormalities on neuroradiological imaging and a pathological cerebrospinal fluid analysis. Due to the unspecific clinical picture and

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Hemophagocytic Lymphohistiocytosis Associated with Malignancies and with Epstein-Barr Virus the overproduction of pro-inflammatory cytokines. HLH can occur as the result of several underlying conditions in which there is excess stimulation of the immune system, in combination with inadequate downregulation of the immune response. Among the stimuli commonly associated with HLH are underlyi

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