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

Sat Mar 11 12:22:54 EST 2017

Dear all, we've recently had a similar case and a moderate response to steroids (2mg/kg and tappering) was observed. Platelet count raised to 45-60.000. 
Hope it helps. 

P. 

Pere Soler Palacín, MD, PhD, MSc. 
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron. 
Vall d'Hebron Research Institute (VHIR) 
Assistant Professor. Universitat Autònoma de Barcelona (UAB) 
Director of the Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies (Barcelona) 
Pg. de la Vall d'Hebron, 119-129 
08035 Barcelona. Spain. 
Tel. 0034934893140 / Fax 0034934893039 
psoler at vhebron.net / 34660psp at comb.cat 
Web: www.upiip.com 
My ORCID 
ResearchGate: http://www.researchgate.net/profile/Pere_Soler-Palacin 
LinkedIn: www.linkedin.com/in/pere-soler-palacin 

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De: "PAGID" <cis-pidd at lists.clinimmsoc.org> 
Per: "CIS-PIDD" <cis-pidd at lyris.dundee.net> 
Enviats: Dissabte, 11 de Març 2017 18:01:37 
Assumpte: [BULK] Re: [cis-pidd] Wiskott Aldrich and Very low platelets 

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 

(Office): 503-494-0829 I (Fax): 503-494-0714 

(E-mail): shereck at OHSU.edu 

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