Hemophagocytic Lymphohistiocytosis (HLH) in Langerhans Cell Histiocytosis (LCH): A Multicenter Retrospective Descriptional Study
نویسندگان
چکیده
منابع مشابه
A case report of hemophagocytic lymphohistiocytosis (HLH).
Hemophagocytic lymphohistiocytosis (HLH), is an uncommon, life-threatening hyperinflammatory syndrome caused by severe hypercytokinemia with excessive activation of lymphocytes and macrophages due to a highly stimulated but ineffective immune process. We report a case of Hemophagocytic Lymphohistiocytosis in a 15 year old boy presenting with fever, lymphadenopathy and pancytopenia due to infect...
متن کاملHemophagocytic lymphohistiocytosis (HLH) and related disorders.
Hemophagocytic lymphohistiocytosis (HLH), which has many genetic causes, is characterized by multi-system inflammation. HLH is a reactive process resulting from prolonged and excessive activation of antigen presenting cells (macrophages, histiocytes) and CD8(+) T cells. Hemophagocytosis, which is mediated through the CD163 heme-scavenging receptor, is a hallmark of activated macrophages/histioc...
متن کاملLangerhans 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 کاملVinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study
BACKGROUND Vinblastine is the standard treatment for children with Langerhans cell histiocytosis (LCH). Whether this treatment could be extended to adults with LCH is questionable. This retrospective multicenter study included 35 adult patients (median age 33 years; 23 men; 80% with multisystem LCH) who were treated with vinblastine + steroids as a first-line chemotherapy and followed for a med...
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...
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ژورنال
عنوان ژورنال: Blood
سال: 2016
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v128.22.707.707