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
You can also use T2* MRI -- normal values are 0.48-2.8 mg/gm dry liver weight and values of >7 mg/gm are associated with severe iron overload. Of note, has been validated for testing of iron in the heart. (See next) 4/n
Value less than 10 ms on cardiac T2* really require intensive therapy to prevent life threatening arrhythmias and more. 5/n
But some of these options are difficult to use (SQUID, T2*, liver biopsy) so people would want to see if a ferritin and LIC have a correlation. This study by Armand et al for leukemia/MDS was hard to draw conclusions based on lower numbers. 6/n
What modalities do people use for treatments? Based on a survey study from 2017 - ppl said they used oral chelation +/- phlebotomy for iron overload in BMT/peds onc patients. Phlebotomy is done if Hgb >11 where you take 5 ml/kg with replacement of fluids. 7/n
Dr. Lalefar says when you do phlebotomy, check LIC every 6-12 months (CBC with each session). Oral iron chelation options include deferasirox (once daily dosing - start 3.5 mg/kg/day and can increase to 7 mg/kg/day - monitor for GI AE, transaminitis, renal tox). 8/n
Another option is deferoxamine (subcut or IV infusion - start at 20 mg/kg over 8 hours 3 nights/week) - Toxicities attached here and how to monitor for these things. 9/n
Is one better than the other? (phlebotomy vs oral chelation for those that are transfusional iron overloaded but no longer are getting transfusions) -- Inati (PBC 2016) said deferasirox had increased benefit in decreasing liver iron -- although you have to consider safety. 10/n
Dr. Lalefar uses this surveillance and treatment algorithm - depending on LIC (liver iron concentration), decides on chelation strategies. #ASPHO2021 11/11 (end!)
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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
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
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
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