Pancreatic Cancer
Brian M. Wolpin, MD, MPH, Editor Although pancreatic cancer remains a leading cause of cancer death, steady progress is being made in understanding the biology of the disease and optimal…
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…
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…
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…
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…
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…
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…
Clinical Characteristics and Treatment of Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH) is a disease caused by clonal proliferation of CD1a+/CD207+ cells that is characterized by a spectrum of varying degrees of organ involvement and dysfunction. Treatment of LCH…
Genomic Alterations in Langerhans Cell Histiocytosis
The discovery of recurrent somatic genomic alterations in Langerhans cell histiocytosis (LCH) has led to a new understanding of LCH as a clonal neoplastic disorder. Most of the abnormalities described…
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…
