[CIS PIDD] [cis-pidd] Wiskott Aldrich and Very low platelets

Sat Mar 11 12:02:02 EST 2017

Dave,

Thanks for your response and abstract. No, he does not respond to platelet
transfusions. We checked a one hour post and his platelet count had not
budged. We gave him 1mg/kg IVIG treating him for auto-immune mediated
thrombocytopenia. We're hoping to take him to transplant in the next month,
I just fear him having a serious bleed until he is ready to go.

Thanks.

-evan

On Sat, Mar 11, 2017 at 8:55 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Dear Evan,
>
>
> There was an abstract by Dr. Bussell awhile ago - see below.  We have used
> Nplate and Promacta in a few kids with XLT and the responses have not been
> that robust.   On the flip side...  we have not run into toxicity.   I
> think they have little downside, but again - the benefit may be minimal.
> In my opinion - I  would not remove the spleen unless there was some very
> compelling reason to do so prior to heading into transplant.   You could
> certainly provide platelet transfusions - does he response to transfusions
> (? 1 hour post-platelet count check).   How much immunoglobulin are you
> using (1 g/kg) ?
>
>
> Best Wishes,
>
>
> Dave
>
>
> David Buchbinder, MD
>
> Division of Hematology
>
> CHOC Children's Hospital
>
>
>
>
> Platelet Function and Response to Thrombopoietin Mimetics In
> Wiskott-Aldrich Syndrome/X-Linked Thrombocytopenia.
> James B. Bussel, Andrew L Frelinger III, William B Mitchell, Mariana P
> Pinheiro, Marc R Barnard, Michael Lampa, Bethan Psaila, Nayla Boulad,
> Hannah Tamary and Alan D. Michelson
> Blood 2010 116:1429
>
>
>
>
> ------------------------------
> *From:* CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> *Sent:* Saturday, March 11, 2017 8:15 AM
> *To:* CIS-PIDD
> *Subject:* [cis-pidd] Wiskott Aldrich and Very low platelets
>
>
> All,
>
> I have a 4 month old male patient with Wiskott-Aldrich. His baseline
> platelet count is about 30-40. He was admitted to our hospital when he was
> 3 months old with RSV and moraxella pneumonia. At that time, his platelet
> count dropped to 2-3. That lasted about a week. As he recovered from his
> infections, his platelets improved.
>
> He was then re-admitted on March 2nd when he was 4 months old- this time
> with Coronavirus and Flu A. Again his platelets have dropped and do not
> really respond to platelet transfusions. He is no longer symptomatic from
> his viral infections, but his platelets have yet to recover. We have given
> him IVIG without any response. I have looked at his blood smear, and his
> platelet count does appear to really be that low.
>
> We are getting him prepared for a bone marrow transplant. In the meantime,
> does anyone have any good recommendations of how to increase his platelet
> count. I am hesitant to take out his spleen. Has anyone tried eltrombopag?
>
> Thanks.
>
> Evan
>
> *Evan Shereck, MD, MEd*
>
> Associate Professor of Pediatrics
>
> Director, Pediatric Hematology/Oncology Fellowship
>
> Block Co-Director, Blood and Host Defense
>
> Oregon Health & Science University I  3181 SW Sam Jackson Park Rd, Mail
> Code: CDRCP  I  Portland, OR 97239
>
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