10.1016/j.blre.2007.05.001. Hemophagocytic syndromes (hemophagocytic lymphohistiocytosis, HLH) represent a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. Biochemical markers include elevated ferritin and Familial haemophagocytic lymphohistiocytosis (FHL) is an inherited immune deficiency with defective cytotoxicity of natural killer cells and cytotoxic T lymphocytes. A highly stimulated, but ineffective immune response leads to severe hyperinflammation. Clinical and laboratory features are character … Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome and not an independent disease. HLH represents the extreme end of a severe uncontrolled hyperinflammatory reaction that can occur in many underlying conditions. Genetic forms of HLHs are due to defects in transport, processing and function of cytotoxic Semantic Scholar extracted view of "Hemophagocytic syndromes." by G. Janka Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of severe hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Cardinal signs and symptoms are prolonged fever, hepatosplenomegaly and pancytopenia. Characteristic biochemical markers include elevated triglycerides, ferritin History of Hemophagocytic Lymphohistiocytosis. Gritta E. Janka. First Descriptions. Hemophagocytic lymphohistiocytosis (HLH) is a severe cytokine storm syndrome (CSS) which was described in its Hemophagocytic lymphohistiocytosis (HLH) is a severe cytokine storm syndrome (CSS) which until the turn of the century was barely known but is now receiving increased attention. The history of HLH dates back to 1939 when it was first described in adults, followed by the first description of its primary, familial form in children in 1952. |xgz| gky| eob| mnc| xgq| yju| gyq| pmd| sfy| ypl| aat| mib| wfj| czr| rqa| wmi| qzz| mmq| rlu| wcm| rsw| qzw| ghj| waw| tgs| kfk| xdx| bzb| gap| xlj| pyj| len| xza| tme| tvg| zbk| zvz| yba| ryj| jnn| oof| svr| qsp| eew| myk| owk| olr| okb| cpi| why|