• Hemophagocytic inflammatory syndrome (HIS) is a rare form of secondary hemophagocytic lymphohistiocytosis caused by an impaired equilibrium between natural killer and cytotoxic T-cell activity, evolving in hypercytokinemia and multiorgan failure. (bvsalud.org)
  • Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDS. (medifind.com)
  • Sintilimab combined with chidamide in the treatment of extranodal nature killer/T-cell lymphoma with secondary hemophagocytic lymphohistiocytosis: Two case reports and literature review. (medifind.com)
  • Limited efficacy of tocilizumab in adult patients with secondary hemophagocytic lymphohistiocytosis: a retrospective cohort study. (medifind.com)
  • More recently, histiocytic diseases have been reclassified into five groups: (1) Langerhans-related, (2) cutaneous and mucocutaneous, (3) malignant histiocytosis, (4) Rosai-Dorfman disease, and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome. (medscape.com)
  • In addition, our program treats children and adolescents with other conditions, including post-transplant lymphoproliferative disorder, hemophagocytic lymphohistiocytosis (HLH), a life-threatening condition caused by immune dysregulation, primarily of histiocytes and lymphocytes, and Langerhans cell histiocytosis (LCH) , which is a cancer-like condition and may involve treatment with chemotherapy . (childrenshospital.org)
  • Histiocytosis UK is dedicated to promoting and funding scientific research into uncovering not only the causes of all histiocytic diseases, which include Langerhans's Cell Histiocytosis and Haemophagocytic Lymphohistiocytosis, but also ensuring early diagnosis, effective treatment and a cure. (histiouk.org)
  • Our doctors provide unparalleled expertise in the diagnosis and treatment of the major types of histiocytosis - Langerhans cell histiocytosis (LCH) and hemophagocytic lymphohistiocytosis (HLH) - and treat rare, related disorders, such as juvenile xanthogranuloma and Rosai-Dorfman disease. (childrenshospital.org)
  • Clinical case of hemophagocytic lymphohistiocytosis: rare or undiagnosed syndrome? (edu.ua)
  • Hemophagocytic lymphohistiocytosis in adults. (edu.ua)
  • A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults. (edu.ua)
  • Genetics and pathogenesis of hemophagocytic lymphohistiocytosis. (edu.ua)
  • The differences between the clinical characteristics and survival time in malignancy- and non-malignancy-associated secondary hemophagocytic lymphohistiocytosis (HLH) are unclear. (biomedcentral.com)
  • Hemophagocytic lymphohistiocytosis (HLH) is characterized by aberrant activation and proliferation of polyclonal CD8 + T lymphocytes and macrophages that infiltrate multiple organs and overproduce inflammatory cytokines [ 1 ]. (biomedcentral.com)
  • On the clinical side, I have developed expertise in the management of patients with neuroblastoma, germ cell tumors, and histiocytic diseases (such as Langerhans cell histiocytosis and hemophagocytic lymphohistiocytosis, or LCH and HLH). (chop.edu)
  • Hemophagocytic Lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome due to pathologic immune activation. (longdom.org)
  • Hemophagocytic lymphohistiocytosis (HLH) is a rare, lifethreatening state of extreme inflammation and tissue destruction. (longdom.org)
  • After consultation with the hematology service, a work-up for hemophagocytic lymphohistiocytosis (HLH) was initiated and the newborn was started on Anakinra prophylactically. (longdom.org)
  • In the majority of patients, this disorder is typically characterized by infantile-onset hemophagocytic lymphohistiocytosis (HLH), which is lethal unless allogeneic transplantation is performed. (biomedcentral.com)
  • The 'accelerated phase' of CHS, namely hemophagocytic lymphohistiocytosis (HLH), develops in 50-85% of patients, and is fatal if not treated. (biomedcentral.com)
  • Broadly speaking, they can be divided into Langerhans and non-Langerhans cell histiocytoses. (thedoctorsdoctor.com)
  • Langerhans cell histiocytoses are all defined by the presence of a unique ultrastructural organelle, the Birbeck granule. (thedoctorsdoctor.com)
  • Currently, the preferred term is Langerhans cell histiocytosis. (thedoctorsdoctor.com)
  • Langerhans cell histiocytosis presenting in the neonatal period: a retrospective case series. (thedoctorsdoctor.com)
  • OBJECTIVES: To describe the morphologic characteristics of skin lesions, extent of extracutaneous disease, and outcomes in patients with neonatal presentation of Langerhans cell histiocytosis (LCH), and to examine clinical predictors of disease prognosis. (thedoctorsdoctor.com)
  • Epidemiologic study of Langerhans cell histiocytosis in children. (thedoctorsdoctor.com)
  • OBJECTIVE: The etiology and pathogenesis of Langerhans cell histiocytosis (LCH) remain poorly understood. (thedoctorsdoctor.com)
  • What Is Childhood Langerhans Cell Histiocytosis? (dana-farber.org)
  • Langerhans cell histiocytosis (LCH) is a rare disorder that occurs when a child has too many of a certain type of cell called Langerhans cells. (dana-farber.org)
  • Langerhans cell histiocytosis may occur at any age, but is most common in young children under 10. (dana-farber.org)
  • How is Langerhans cell histiocytosis diagnosed? (dana-farber.org)
  • What are Langerhans cell histiocytosis treatment options? (dana-farber.org)
  • [ 2 ] For example, the entity now referred to as Langerhans cell histiocytosis (LCH) was initially divided into eosinophilic granuloma, Hand-Schüller-Christian disease, and Abt-Letterer-Siwe disease, depending on the sites and severity. (medscape.com)
  • Most recently, this designation was changed to Langerhans cell histiocytosis based on the suggestion by Nezelof that the Langerhans cell represented the primary cell involved in the pathophysiology of the disease. (medscape.com)
  • [ 5 , 6 ] Although several histiocytic disorders are briefly discussed in this article (see History ),the primary focus is on Langerhans cell histiocytosis. (medscape.com)
  • Pilot study to estimate the safety and effectiveness of hydroxyurea and methotrexate recurrent langerhans cell histiocytosis (LCH-HU-pilot). (medifind.com)
  • Current perspectives on the role of liver transplantation for Langerhans cell histiocytosis: A narrative review. (medifind.com)
  • Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the presence of cells with characteristics similar to bone marrow-derived Langerhans cells juxtaposed against a backdrop of hematopoietic cells, including T-cells, macrophages, and eosinophils. (medscape.com)
  • The term Langerhans cell histiocytosis is generally preferred to the older term, histiocytosis X. This newer name emphasizes the histogenesis of the condition by specifying the type of lesional cell and removes the connotation of the unknown ("X") because its cellular basis has now been clarified. (medscape.com)
  • The pathogenesis of Langerhans cell histiocytosis (LCH) is unknown. (medscape.com)
  • Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. (msdmanuals.com)
  • Pulmonary Langerhans Cell Histiocytosis Pulmonary Langerhans cell histiocytosis (PLCH) is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. (msdmanuals.com)
  • Langerhans cell histiocytosis (LCH) is a dendritic cell (antigen-presenting cell) disorder. (msdmanuals.com)
  • Symptoms and signs of Langerhans cell histiocytosis vary considerably depending on which organs are infiltrated. (msdmanuals.com)
  • An International team of Researchers including researchers from Newcastle, Dr Paul Milne and Prof Matthew Collin have made progress in understanding how the childhood cancer Langerhans Cell Histiocytosis (LCH) damages tissue in the body. (histiouk.org)
  • This work represents an important advance in understanding how the lesions of Langerhans Cell Histiocytosis develop. (histiouk.org)
  • When asked "What does it mean for our paediatric patients of Langerhans Cell Histiocytosis? (histiouk.org)
  • Histiocytosis X was renamed Langerhans-cell histiocytosis , reflecting the concept that LCH cells represented dysfunctional epidermal Langerhans cells. (capsulehealth.one)
  • Available at: http://www.cancernetwork.com/oncology-journal/langerhans-cell-histiocytosis-emerging-insights-and-clinical-implications [Accessed 1 Jul. (capsulehealth.one)
  • Patients appear to have normal lipid metabolism and are classified as a normolipemic non-Langerhans cell histiocytosis. (nih.gov)
  • Ruxolitinib as an effective treatment for panniculitis associated hemophagocytic syndrome: A report of 2 cases and literature review. (medifind.com)
  • Hemophagocytic syndrome that is associated with viral infection. (nih.gov)
  • The use of cyclosporin-A in the treatment of virus-associated hemophagocytic syndrome in adults. (nih.gov)
  • Analysis of prognostic risk factors in children with Epstein-Barr virus-associated hemophagocytic syndrome. (nih.gov)
  • Virus-associated hemophagocytic syndrome as a major contributor to death in patients with 2009 influenza A (H1N1) infection. (nih.gov)
  • Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. (nih.gov)
  • Early Use of Blood Purification in Severe Epstein-Barr Virus-Associated Hemophagocytic Syndrome. (nih.gov)
  • Epstein-Barr virus-associated hemophagocytic syndrome. (nih.gov)
  • Fulminant Epstein-Barr virus-associated hemophagocytic syndrome in a renal transplant patient and review of the literature. (nih.gov)
  • The term MAS originally referred to a hemophagocytic syndrome (HPS) variant that occurs in patients with autoinflammatory and autoimmune diseases, particularly systemic onset juvenile rheumatoid arthritis and adult-onset Still's disease. (biomedcentral.com)
  • Background: Adult hemophagocytic syndrome is a critical condition that is often difficult to diagnose and results in a bad prognosis because of the lack of effective and unified treatment. (scirp.org)
  • Aim: To investigate the clinical, diagnosis and treatment of hemophagocytic syndrome. (scirp.org)
  • The results confirmed the diagnosis of hemophagocytic syndrome related to EB (Epstein-Barr) virus infection. (scirp.org)
  • Hemophagocytic syndrome (HS) is a clinically rare autoimmune disease that is characterized in clinical practice by fever, hepatomegaly, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow, liver, spleen, and lymph nodes. (scirp.org)
  • They can be functional, exhibiting a hormonal hypersecretion syndrome, but can be non-functional presenting with non-specific symptoms and include insulinoma, glucagonoma, VIPoma, somatostatinoma (SSoma), PPoma and Zollinger-Ellison syndrome (ZES, or gastrinoma) and other ectopic hormone producing tumors (such as GRFoma) (see these terms). (findzebra.com)
  • Primary HLH mainly includes Familial Hemophagocytic Lympho Histiocytosis (FHLH) and immunodeficiency syndrome associated HLH. (ommegaonline.org)
  • Hemophagocytic syndrome (HPS) is clinically defined as a combination of fever, liver dysfunction, coagulation abnormalities, pancytopenia, progressive macrophage proliferation throughout the reticuloendothelial system, and cytokine over-production, and may be primary or secondary to infectious, auto-immune, and tumoral diseases. (biomedcentral.com)
  • Hemophagocytic syndrome (HPS) is a potentially fatal condition due to dysregulated lymphocyte activation and proliferation, mainly characterised by impaired or inactive natural killer (NK) cells and cytotoxic T cells, which leads to macrophage hyperactivation and over-expression of cytokines [ 1 ]. (biomedcentral.com)
  • The syndrome was first described in 1939 as poorly-controlled histiocyte proliferation, but has since also been called hemophagocytic histiocytosis and macrophage activation syndrome [ 5 - 7 ]. (biomedcentral.com)
  • Another fatality has been reported in which Kikuchi disease appeared concurrently with SLE and was complicated with hemophagocytic syndrome and severe infection. (medscape.com)
  • The working group of the Histiocyte Society divided histocytic disorders into three groups: (1) dendritic cell histiocytosis, (2) macrophage-related disorders, and (3) malignant histiocytosis. (medscape.com)
  • Each category of histiocytosis can be traced to reactive or neoplastic proliferation in one of these cell lineages. (medscape.com)
  • although, in terms of nomenclature, lymphoproliferative disorders are a subclass of immunoproliferative disorders-along with hypergammaglobulinemia and paraproteinemias. (wikipedia.org)
  • The histiocytoses encompass a group of diverse disorders characterized by the accumulation and infiltration of variable numbers of monocytes, macrophages, and dendritic cells in the affected tissues. (medscape.com)
  • At the very heart of the Histiocytosis Association is the steadfast commitment to finding a cure for histiocytic disorders. (histio.org)
  • The Histiocytosis Association's Annual Research Grant Program funded research projects across histiocytic disorders every year, based on the generosity of the community. (histio.org)
  • The Dana-Farber/Boston Children's Cancer and Blood Disorders Center provides expert treatment for newly diagnosed or recurrent histiocytosis, which is a cancer-like condition and may involve treatment with chemotherapy . (childrenshospital.org)
  • Long-term safety and clinical outcomes of olipudase alfa enzyme replacement therapy in pediatric patients with acid sphingomyelinase deficiency: two-year results. (medifind.com)
  • The program sees more than 125 newly diagnosed patients with leukemia and lymphoma annually, and continues to lead high-impact clinical trials designed to increase cure rates, decrease treatment-related toxicities, and improve care for long-term survivors. (childrenshospital.org)
  • The Histiocytosis Association Research Grant Program operates very much on a "parallel highway" - funding grants for laboratory research, financially supporting clinical trials, and managing and participating in the evolution of the Histiocyte Society . (histio.org)
  • HS is often described in binary terms as "primary," indicating Mendelian inheritance of gene mutations resulting in cytotoxic lymphocyte dys- function, or "secondary" indicating an acquired reactive disorder. (scirp.org)
  • The charity focuses on histiocytosis in babies, children, young people and adults. (histiouk.org)
  • However, in recent reports on HPS in adults, the term MAS is often used almost interchangeably with acquired HPS. (biomedcentral.com)
  • A deeper knowledge of immunologic patterns contributing to HIS pathogenesis in ADA-SCID patients is desirable, to identify new targeted treatments and ensure patients' long-term recovery. (bvsalud.org)
  • 2018) Valoración de un nuevo ensayo quimioluminiscente en comparación con ELISA en la detección de IgG contra el virus de la hepatitis E. Revista de la Sociedad Andaluza de Microbiología y Parasitología Clínica. (vircell.com)
  • The term encompasses both the medical specialty which uses tissues and body fluids to obtain clinically useful information, as well as the related scientific study of disease processes. (bionity.com)
  • Recurrence of anti-MDA5 antibody-positive clinically amyopathic dermatomyositis after long-term remission: A case report. (kyoto-u.ac.jp)
  • Two decades later, with the advent of electron microscopy, Nezelof and colleagues identified a unique intracellular organelle, the Birbeck granule , in histiocytosis X lesions. (capsulehealth.one)
  • Minimally Invasive Resection of Benign Osseous Tumors of the Spinal Column: 10 Years' Experience and Long-Term Outcomes of a Specialized Center. (medifind.com)
  • Glycogenosis due to glucose-6-phosphatase deficiency (G6P) type b, or glycogen storage disease (GSD) type 1b, is a type of glycogenosis due to G6P deficiency (see this term). (nih.gov)
  • In addition to treating lymphomas and leukemias, we treat the primary types of histiocytosis, which is not a form of cancer but shares some common characteristics and is often treated using some of the same therapies. (childrenshospital.org)
  • The Histiocytosis Association Research Grant Program was established in 1990 by the Association's Board of Trustees with the objective of identifying and funding the most important and promising research initiatives - those that will lead to more effective treatments and, ultimately, a cure. (histio.org)
  • By subscribing, I agree to MediFind's Terms & Conditions and Privacy Policy . (medifind.com)
  • Histiocytosis is a group of conditions that involve too many white blood cells (histiocytes) in your child's blood. (childrensnational.org)
  • Hemophagocytic lymphohistocytosis in trisomy 21: successful treatment with interferon inhibition. (medifind.com)
  • STUDY DESIGN: We used a case-control study design to obtain data from parents of children with LCH (n = 459) who were members of the Histiocytosis Association of America and Canada. (thedoctorsdoctor.com)
  • We present the case of a critically ill newborn full-term via urgent cesarean delivery for concerns for hydrops fetalis, ultimately found to have familial HLH. (longdom.org)