The Role of Hematopoietic Stem Cell Transplantation in Treatment of Hemophagocytic Lymphohistiocytosis

Mar 1, 2017 by in HEMATOLOGY Comments Off on The Role of Hematopoietic Stem Cell Transplantation in Treatment of Hemophagocytic Lymphohistiocytosis

The role of reduced-intensity allogeneic hematopoietic stem cell transplantation (HSCT) from a variety of donor sources in improving survival for children with familial hemophagocytic lymphohistiocytosis (HLH) is well-documented. The heterogeneity…

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Macrophage Activation Syndrome

Mar 1, 2017 by in HEMATOLOGY Comments Off on Macrophage Activation Syndrome

Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic disorders that occurs most commonly in systemic juvenile idiopathic arthritis. In recent years, there have been several advances in…

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Hemophagocytic Lymphohistiocytosis in Adults

Mar 1, 2017 by in HEMATOLOGY Comments Off on Hemophagocytic Lymphohistiocytosis in Adults

Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal syndrome of pathologic immune dysregulation characterized by clinical signs and symptoms of extreme inflammation. HLH can occur as a genetic or…

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Pathogenesis of Hemophagocytic Lymphohistiocytosis

Mar 1, 2017 by in HEMATOLOGY Comments Off on Pathogenesis of Hemophagocytic Lymphohistiocytosis

Hemophagocytic Lymphohistiocytosis (HLH), an inherited life-threatening inflammatory disorder, has gained growing recognition not only in children but also increasingly in adults over the past 2 decades. HLH involves inborn defects…

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Familial Hemophagocytic Lymphohistiocytosis

Mar 1, 2017 by in HEMATOLOGY Comments Off on Familial Hemophagocytic Lymphohistiocytosis

Familial hemophagocytic lymphohistiocytosis (FHL) is a rare heritable disorder of immune regulation that is typically characterized by sudden onset of severe systemic illness. Functional impairment or absence of 1 or…

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Strategies for the Prevention of Central Nervous System Complications in Patients with Langerhans Cell Histiocytosis

Mar 1, 2017 by in HEMATOLOGY Comments Off on Strategies for the Prevention of Central Nervous System Complications in Patients with Langerhans Cell Histiocytosis

Diseases of the central nervous system (CNS) are common in patients with Langerhans cell histiocytosis (LCH). Besides active LCH lesions, neurodegenerative (ND) lesions of the cerebellum and/or basal ganglia may…

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Cell(s) of Origin of Langerhans Cell Histiocytosis

Mar 1, 2017 by in HEMATOLOGY Comments Off on Cell(s) of Origin of Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is heterogeneous disease characterized by common histology of inflammatory lesions containing Langerin + (CD207) histiocytes. Emerging data support a model in which MAPK activation in self-renewing…

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