histiocytosis

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Related to Lymphohistiocytosis: Histiocytes

histiocytosis

[‚his·tē·ō‚sī′tō·səs]
(medicine)
Abnormal proliferation of histiocytes, especially in hematopoietic tissues.
McGraw-Hill Dictionary of Scientific & Technical Terms, 6E, Copyright © 2003 by The McGraw-Hill Companies, Inc.
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References in periodicals archive ?
Hemophagocytic lymphohistiocytosis: review of etiologies and management.
Hemophagocytic Lymphohistiocytosis in Dermatomyositis.
Hemophagocytic lymphohistiocytosis in a rheumatoid arthritis patient treated with infliximab.
Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol 2015; 90:220-224.
Haemophagocytic lymphohistiocytosis (HLH) is a rare disease with a sepsis-like progression that leads to multiple organ dysfunction syndrome, especially in preterm infants.
Macrophage activation syndrome (MAS), also known as hemophagocytic lymphohistiocytosis (HLH), is a rare and potentially fatal complication of several autoimmune diseases, including juvenile idiopathic arthritis and SLE.
Although most patients with infectious mononucleosis (IM) recover without major clinical sequelae, a number of harmful complications can occur, including hepatitis, splenic rupture, and hematological abnormalities [1]; among the latter, the hemophagocytic lymphohistiocytosis (HLH) is particularly harmful because it is characterized by the massive production and release of inflammatory mediators caused by the uncontrolled activation of T-lymphocytes, NK-cells, and macrophages; the ensuing clinical picture resembles septic shock and can determine a multiple organ dysfunction syndrome (MODS) [2-4].
Therefore, pathogenicity is unchecked, leading to uncontrolled lymphoproliferation and severe manifestations including fulminant infectious mononucleosis and hemophagocytic lymphohistiocytosis (HLH).
Hemophagocytic lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome characterized by cytopenias with fevers, chills, malaise, myalgias, and diaphoresis with high morbidity and mortality [1].