Up next - Update on TMA! (thrombotic microangiopathy) at #ASPHO2021 Thread here 1/n
Transplant associated-TMA (TA-TMA) defined here. Look at the labs that you should use to help guide this diagnosis. High risk criteria of TA-TMA on second slide. 2/n
TA-TMA have a really high morbidity with a lot of multiorgan injury (intestinal, pulmonary, CNS)... scary -- I don't see this often (since I am no longer working in transplant patients) but the information can be translatable to other TMA type processes. 3/n
I need this reminder all the time as I continue to learn about this -- complement. Terminal complement activation is measured by elevations in cC5b-9. C5 blockage (eculizumab) is treatment for TA-TMA. With this 1 yr OS from 16.7 to 66% w/ this therapy. 4/n
When it's not working - think about high eculizumab clearance, high complement generation when there's a lot of inflamm (esp with GI-TMA) -- so think about personalized PK for these patients and know that you have to follow complement levels (CH50 and C5b9) to resolution. 5/n
Dr. Jodele's summary here for risk stratification and treatment for TA-TMA. Thanks for sharing at #ASPHO2021 - a good reminder for me about complement inhibition! 5/5
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Dr. Sidonio reviewing treatment decisions re: inhibitor patients in the #emicizumab era at #ASPHO2021 - starting with a reminder about bypassing agents and their unique dosing 1/n
ITI (high dose factor exposure) is the standard for getting rid of inhibitors -- worse success if historical high peak titre, time since dx of inhibitor, etc. 2/n
But despite that it's expensive, burdensome... all the major organizations nationally and internationally say you should attempt it! 3/n
Now for an update on #TTP -- always a disease I enjoy teaching about -- the pathophysiology combines so much #heme goodness. Also, I always enjoy the summary slide up front from Dr. George. #ASPHO2021 1/n
Why is this important? Because patients with sickle cell disease have a decrease life expectancy! (by 30 years!) Not only that -- Dr. Allison King found that patients with #sicklecell saw that those on Medicaid clearly repeated grade levels. #ASPHO2021 2/n
From the SIT trial - silent cerebral infarcts wasn’t the risk factor.. per capita income was! Look at the OR of 6.4 for those in the lowest quartile. The most at risk are our older males. #ASPHO2021 3/n
Management of iron overload for #phodocs to pay attention to. Talk by Dr. Lalefar from @UCSFBenioffOAK. Follow this thread 1/n
Who should be monitored for iron overload? Those that have a transfused volume >100 ml/kg (~10 transfusions) #ASPHO2021 2/n
Dr. Lalefar suggests checking ferritin, but remember to check with other iron studies. She says a serum ferritin >1000 ug/L is the common threshold used. (But this was based on correlation studies with liver iron from mostly Caucasian hemochromatosis patients) 3/n
Peak risk of catheter associated DVT at 4 days after catheter insertion. The CRETE trial used ppx enoxaparin on day one (target 0.2-0.5 anti-Xa level) and showed may be able to prevent DVT. 1/n
But amongst infants (child <1 year) no real change in risk - but older children have risk reduction from Dr. Faustino's CRETE trial. He suggests due to differences in size of line versus vein size (infants have less delta) 2/n
Infants have lower thrombin generation which may be why ppx enoxaparin may not help them as much -- they then thought about trying therapeutic enoxaparin in infants to prevent CA-DVT. 3/n
Snapshot of current #phodocs peds data on doac studies. Rivaroxaban (einstein jr) and Dabigatran completed.. data to follow in this thread #ASPHO2021 1/n
Prelim data from rivaroxaban showed <12 kg patients needed TID dosing. The phase 3 dosing is given here. Phase 3 data showed decreased rate of VTE and no major bleeding. 2/n
Dabigatran (DIVERSITY) data was published Jan 2021 phase 2b/3 non-inferiority trial. They used composite primary endpoint of complete resolution, freedom from recurrent VTE and VTE-related death. 3/n