Hemophagocytic lymphohistiocytosis and primary immune deficiency disorders.


Hemophagocytic lymphohistiocytosis (HLH) is characterized by uncontrolled immune activation and is traditionally associated with inherited gene defects or acquired causes. In addition to abnormalities in cytotoxic granules and lysosomes, various primary immune deficiency disorders (PID) have been identified among patients suffering from HLH. Our purpose was… (More)
DOI: 10.1016/j.clim.2014.09.008


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