Thursday, July 15, 2010


Is there evidence that Plasma Excahnge or pheresis works in HUS/TTP or TMA of pregnancy?
This is hard to define as TMA during pregnancy can be from many causes, APLAS syndrome, ADAMTS 13 inhibitor mediated, pre eclampsia, HELLP syndrome and Severe HTN mediated.
First and foremost, 20% of it can happen Post partum, need to rule out APLAS syndrome as well

Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome complicating pregnancy is associated with high maternal mortality and long-term morbidity. Preterm delivery and intrauterine fetal death are frequent complications of these pregnancies. Improved survival after this disorder has been attributed to aggressive treatment with plasma transfusion or plasmapheresis.If one knows that the ADAMTS13 inhibitor is positive, TPE will be of some help. Otherwise what is the evidence for the rest. well, there is only one paper( case series that looks at this). It talks about  pre and post partum TTP and its a case series of 11 patients and TPE did show some survival benefit. I have listed the reference below.
So at this point, one would treat with TPE and send of ADAMTS 13 levels and inhibitor levels and continue TPE if there is an inhibitor for sure. But do we continue TPE if this is severe HTN or pre eclampsia induced TMA, probably not?
At this point, don't think there is much evidence for TPE but that's all we have. More work needs to be done in this area.


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