histiocytosis/l tyrosin

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Tyrosine phosphatase SHP-1 is expressed higher in multisystem than in single-system Langerhans cell histiocytosis by immunohistochemistry.

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Langerhans cell histiocytosis (LCH) is a proliferative disorder of Langerhans cell (LC)-like CD1a-positive cell (LCH cell) with unknown causes. LCH consists of two subtypes: single-system LCH (LCH-SS) with favorable prognosis and multisystem LCH (LCH-MS) with poor prognosis. LCH has been indicated

Langerhans cell histiocytosis with central nervous system involvement--complete response to 2-chlorodeoxyadenosine after failure of tyrosine kinase inhibitor therapies with sorafenib and imatinib.

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Langerhans cell histiocytosis (LCH) is rare in adults, and only a subset of these patients suffers from central nervous system (CNS) involvement. Hence, evidence-based treatment recommendations are lacking. A case of a 20-year-old student with multisystem LCH and extensive CNS involvement is

Immunohistochemical and molecular cytogenetic evaluation of potential targets for tyrosine kinase inhibitors in Langerhans cell histiocytosis.

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Langerhans cell histiocytosis is a rare disorder of Langerhans cells, a component of the dendritic cell system, with an unknown pathogenesis. Conventional therapy for patients with Langerhans cell histiocytosis is usually effective, but some patients are refractory to treatment or develop toxicity.

Abnormal serum phenylalanine-tyrosine ratio and hyperferritinemia in malignant histiocytosis.

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Nine cases of childhood malignant histiocytosis (MH) showed an abnormally high serum phenylalanine (Phe)/tyrosine (Tyr) ratio (3.47 +/- 1.32) coincident with hyperferritinemia (50,800 +/- 33,600 ng/ml). Lactate dehydrogenase activity was also increased in these patients. These values were compared

[Diagnostic utility of tyrosine hydroxylase in peripheral neuroblastic tumors].

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Objective: To investigation the diagnostic utility of tyrosine hydroxylase (TH) immunohistochemically as a marker of peripheral neuroblastic tumors(pNT). Methods: The study included 1 024 cases, 643 primary and metastatic pNT cases, 381 non-pNT cases, including small round cell tumors such as

Langerhans cell histiocytosis in children: from the bench to bedside for an updated therapy.

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Langerhans cell histiocytosis (LCH) is a rare disease, affecting subjects of any age, with extremely variable clinical manifestations. Although most patients with LCH have localized disease, requiring local or even no therapy, those patients with disseminated, 'multi-system' disease require specific

Abnormal cell surface antigen expression in individuals with variant CD45 splicing and histiocytosis.

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Hemophagocytic lymphohistiocytosis (HLH) and Langerhans cell histiocytosis (LCH) are members of a group of rare heterogenous disorders, the histiocytoses, characterized by uncontrolled accumulation of pleomorphic infiltrates of leukocytes. The etiology of these diseases is mainly unknown. CD45 is a

Increased blood myeloid dendritic cells and dendritic cell-poietins in Langerhans cell histiocytosis.

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Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is a reactive proliferative disease of unknown pathogenesis. Current therapies are based on nonspecific immunosuppression. Because multiple APCs, including Langerhans cells and macrophages, are involved in the lesion

Interleukin-1 loop model for pathogenesis of Langerhans cell histiocytosis.

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We propose Langerhans cell histiocytosis (LCH) is an inflammatory process that is prolonged by mutations. We hypothesize that Merkel cell polyomavirus (MCPyV) infection triggers an interleukin-1 (IL-1) activation loop that underlies the pathogenesis of LCH. Langerhans cells (LCs) are antigen

Mutations in Receptor Tyrosine Kinases Drive Histiocytic Disorders.

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Genomic analysis identified previously unknown potential histiocytosis-driving mutations.

Activating mutations in CSF1R and additional receptor tyrosine kinases in histiocytic neoplasms.

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Histiocytoses are clonal hematopoietic disorders frequently driven by mutations mapping to the BRAF and MEK1 and MEK2 kinases. Currently, however, the developmental origins of histiocytoses in patients are not well understood, and clinically meaningful therapeutic targets outside of BRAF and MEK are

Clinical outcome, PDGFRβ and KIT expression in feline histiocytic disorders: a multicentre study.

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Information about histiocytic disease in cats is limited. The aim of this study was to document clinical findings and outcome in feline histiocytic disorders, and characterize the expression of PDGFRβ and KIT in order to identify potential treatment targets. Morphologically diagnosed feline

Anaplastic lymphoma kinase: role in cancer pathogenesis and small-molecule inhibitor development for therapy.

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Anaplastic lymphoma kinase (ALK), a receptor tyrosine kinase in the insulin receptor superfamily, was initially identified in constitutively activated oncogenic fusion forms - the most common being nucleophosmin-ALK - in anaplastic large-cell lymphomas, and subsequent studies have identified ALK

A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy.

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BACKGROUND Rosai-Dorfman disease is a non-Langerhans cell histiocytosis that recently has been treated successfully with imatinib mesylate in a patient with a systemic variant of the disease. METHODS We describe a 69-year-old man with cutaneous Rosai-Dorfman disease manifesting as progressive,

Idiopathic central diabetes insipidus in children and young adults is commonly associated with vasopressin-cell antibodies and markers of autoimmunity.

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OBJECTIVE Autoimmune targeting of hypothalamic-neurohypophyseal structures in children and young adults with posterior pituitary and anterior pituitary dysfunction, as well as pituitary stalk involvement, are not yet completely understood. METHODS We aimed to (1) evaluate the presence of circulating

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