Wednesday, October 2, 2013

CONSULT ROUNDS: Hemophagocytic syndrome and the kidney?

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. 

Renal manifestations:

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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