Hemophagocytic Lymphohistiocytosis (HLH)

Started by PICUfellow
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PICUfellow

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Hi guys,

Are there any hematologists or hematology fellows reading who can explain the exact/specific causes of the following in HLH:

-Hyperferritinemia
-Hypertriglyceridemia
-Increased LDH
-Hypofibrinogenemia
 
High circulating cytokines characteristic of HLH: IL1, TNFalpha, IFNgamma, IL6, IL2Ralpha.

-Hyperferritinemia
Ferritin secreted by activated macrophages and hepatic acute phase reactant.

-Hypertriglyceridemia
High TNFalpha inhibits lipoprotein lipase enzymatic activity.

-Increased LDH
Not sure if due to hepatic tissue injury by infiltrating lymphocytes/histiocytes or if there is some intravascular red cell hemolysis. LDH isoenzymes helpful.

-Hypofibrinogenemia
Plasminogen activator secreted by activated macrophages leading to excess fibrinolysis.
 
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