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

[CIS-PIDD]

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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