Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease of the lymphatic and reticuloendothelial system that can be inherited (primary or familial HLH) or acquired (secondary HLH) [1]. Both inherited and acquired HLH are often triggered by infections. The clinical picture is reminiscent of sepsis including fever, splenomegaly, and tricytopenia. Further characteristics are elevated concentration of serum liver enzymes, triglycerides, ferritin, and cytokines (especially tumor necrosis factor alpha) as well as decreased fibrinogen levels [2]. Biopsy of the bone marrow and other organs typically shows erythrophagocytosis by hyperactive macrophages (histiocytes). Recent studies in familial HLH suggest that abnormal macrophage activation results from impaired lymphocyte-mediated cytotoxicity and defective triggering of apoptosis [3].
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Tiede, A., von Depka Prondzinski, M., Kreipe, H.H., Wagner, A., Ganser, A., Heil, G. (2004). Hyperfibrinolysis in Hemophagocytic Lymphohistiocytosis. In: Scharrer, I., Schramm, W. (eds) 33rd Hemophilia Symposium. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-18260-0_28
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DOI: https://doi.org/10.1007/978-3-642-18260-0_28
Publisher Name: Springer, Berlin, Heidelberg
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