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In hematology, hemophagocytic lymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis (British spelling), and hemophagocytic or haemophagocytic syndrome, [1] is an uncommon hematologic disorder seen more often in children than in adults.
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by a systemic inflammatory or, in extreme cases, overwhelming cytokine storm condition. It is due to the pathological proliferation and activation of benign histiocytes , macrophages , and lymphocytes along with the excessive release of proinflammatory cytokines by these ...
Micrograph showing hemophagocytosis in the spleen. H&E stain.. Hemophagocytosis is a dangerous form of phagocytosis in which histiocytes engulf red blood cells, white blood cells, platelets, and their precursors [1] in bone marrow and other tissues.
X-linked lymphoproliferative disease; Other names: Duncan disease, Purtilo syndrome: Specialty: Hematology : Symptoms: Reduced resistance to the Epstein-Barr virus (EBV), leading to infectious mononucleosis, hemophagocytic lymphohistiocytosis (HLH), dysgammaglobulinemia, non-Hodgkin lymphoma, aplastic anemia, vasculitis, chronic gastritis, skin lesions
Lymphoproliferative disorders are a set of disorders characterized by the abnormal proliferation of lymphocytes into a monoclonal lymphocytosis.The two major types of lymphocytes are B cells and T cells, which are derived from pluripotent hematopoietic stem cells in the bone marrow.
It is thought to be closely related and pathophysiologically very similar to reactive (secondary) hemophagocytic lymphohistiocytosis (HLH). [1] The incidence of MAS is unknown as there is a wide spectrum of clinical manifestations, and episodes may remain unrecognized.
Lymphohistiocytosis is a similar immune system disease characterized by the inappropriate activation of natural killer cells, CD8+ cytotoxic T-cells, and macrophages, involving principally the liver, spleen and central nervous system and associated with severe lymphoid atrophy.
They can become hemophagocytes. Once considered the diagnostic hallmarks of macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH), they can be abundant in organs of the reticuloendothelial system during systemic inflammation.