hemophagocytic lymphohistiocytosis การใช้

• HLH I suppose is Hemophagocytic lymphohistiocytosis, but what could NEMO be?
• Perforin deficiency results in the commonly fatal disorder familial hemophagocytic lymphohistiocytosis ( FHL or HLH ).
• Nearly half of the cases of type 2 familial hemophagocytic lymphohistiocytosis are due to bi-allelic PRF1 mutations.
• Some sources recommend classifying this condition as " X-linked familial hemophagocytic lymphohistiocytosis " instead of X-linked lymphoproliferative disease.
• Adult-onset Still's disease, some porphyrias, and hemophagocytic lymphohistiocytosis / macrophage activation syndrome are diseases in which the ferritin level may be abnormally raised.
• The differential diagnosis of HLH includes secondary HLH and macrophage-activation syndrome or other primary immunodeficiencies that present with hemophagocytic lymphohistiocytosis, such as X-linked lymphoproliferative disease.
• Hemophagocytic lymphohistiocytosis ( HLH ) is characterized by excessive engulfment of hematopoietic stem cells ( HSCs ) by bone marrow macrophages, and this has been found to result from down regulation of CD47 expression on HSCs, enabling macrophages to eat them alive.
• Cullen further states in his paper that granzymes may have a role in immunomodulation, or the job of maintaining homeostasis in the immune system during an infection .  In humans, loss of perforin function leads to a syndrome called familial hemophagocytic lymphohistiocytosis [ & ] .