Thursday, July 8, 2010


Old article, but nice review:
NEJM 2006;354:1927-1935

Key Points:

-90% mortality of TTP if untreated
-Pentad not needed for dxn, only MAHA and low plts along with appropriate clinical setting
-Childhood HUS with diarrheal prodrome (Shiga toxin/E.Coli O157:H7) treatment is only supportive care and antiobiotics should be withheld intially unless toxic/bacteremic since it may release more Shiga toxin and exacerbate the HUS
-ADAMTS13 deficiency (cleaving ulVWF) NOT seen in diarrheal prodrome HUS
-Neuro symtpoms more with TTP, renal more with HUS
-Distinguish TMA due to other disorders incl malignant HTN and autoimmune disorders
-LDH levels will be sky high often, schistos, polychromatophilic red cells, indirect bili, neg direct Coombs
-Acute flares may or may not have low levels of ADAMTS13, so cannot go by this, it only tells you risk of relapse
-PEX!!!! If delay, can use plasma infusion but not as effective. Steroids maybe
-Causes: Idiopathic (rare to have renal manifestations), pregnancy, autoimmune d/o, diarrheal prodrome (HUS), immune mediated and dose dependant drug toxicity, HST (TMA usually limited to kidney, unlikely to benefit from PEX)
-PEX works even if no severe ADAMTS13 deficiency


  1. Nice review Ojas. I call these diseases more of a TMA rather than HUS/TTP. I think that HUS and TTP are two extremes of a TMA process. And if we rely on those diagnosis, we can miss subtle TMAs.
    And in terms of TPE response, I think that an ADAMTS13 inhibitor related TMA is the only one that will respond to TPE and perhaps a TMA related to an anther autoantibody like in SLE. Rest of which are non antibody mediated, will less likely respond to TPE.


    Check out the latest blog from Dr.Lane about HUS as well.


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