Recently, we wrote an article on Bone marrow transplant or HSCT related TMA for AJKD.Few things that we have seen in our experience and also what we learnt while writing on this topic is important for Nephrologists to understand.
1.
Diagnosis of TMA related to HSCT that effects
the kidney is hard to diagnosis. It is likely the most common kidney biopsy
finding post HSCT. Besides the lab
parameters of kidney injury, TRENDING the LDH, haptoglobin, platelets and
hemoglobin is critical. In addition, HTN might be the first and most important
sign of impending TMA. If the patient requires more than 2 meds for HTN
control, one for CNI and other for steroids, it is possible that there is a
smoldering TMA . Perhaps we miss some of these cases due to this. Early
Nephrology referral might be key as urinalysis is not uniformly done in most
centers years post HSCT.
2.
Once infections such as parvo, BK and CMV have
been ruled out, a complement mediated process is the likely cause.
3.
CNIs are usually portrayed as the most likely
culprit but not all allogenic HSCTS are on CNI and none of the autologous HSCT
are on CNIs, yet TMA ensues. In many instances, TMA still gets worse.
4. Based on some recent findings in the
basic science world and few case reports, we propose that TMA following HSCT in many cases
might be an “endothelial” variant of GVHD. Treating the underlying GVHD might
be the best option in these cases. This might open certain treatment avenues
that we haven’t really encountered.
5.
If the TMA is not a ADAMTS13 process, or a “antibody”
being removed, TPE doesn’t really help in most cases. Rituximab can be a
potential option as this might also help treat GVHD. Alternatively, in many
pediatric patients, eculizumab has been used with some success to halt the
activated complement process.
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