What is hemophagocytic syndrome? and what do nephrologists have to do with it?
Its a dysregulation and inappropriate activation of the immune system. Its infiltration of non malignant macrophages and phagocytosis of blood cells.
Key features: fevers, hepatosplenomegaly, pancytopenia, low fibrinogen levels, LFTS dysfunction, seizures, hypertriglyceridemia. and multi organ failure with AKI
Primary cause: immune dysregulation
Secondary causes: autoimmune such as Stills disease, SLE. Infections such as EBV. Herpes, PB19, HIV.etc and malignancy such as T and B leukemias/lymphomas.
ATN and interstitial disease( being most common on autopsy findings), macrophage infiltration, intra renal hemaphagocytosis
Collapsing FSGS( most common glomerular disease), MCD, FSGS
TMA, and intravascular lymphoma
Chemotherapy usually etoposide based
EBV disease- think this syndrome as well if above features are present.
What do nephrologists have to do with it?- recognize it when appropriate as AKI and proteinuria is not uncommon with this entity.
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