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

[CIS-PIDD]

Dear Evan,
We are treating a WAS-like (WIP deficient) patient with romiplostim with moderate success, >40-60k platelets and no bleedings. We tried eltrombopag but it didn't work so well due to short bowel syndrome after surgical resection of volvolus and probably low resorption. Please contact me for more details,
Best
Markus

Markus G. Seidel, M.D. | Professor of Translational Pediatric Hematology and Immunology | Div. of Pediatric Hematology-Oncology | Dept. of Pediatric and Adolescent Medicine | Medical University Graz | Auenbruggerpl. 34/2 | A-8036 Graz | Austria | T. 0043 316 385 80215<tel:0043%20316%20385%2080215>| F. 0043 316 385 13717<tel:0043%20316%20385%2013717> | Secr. 0043 316 385 13485<tel:0043%20316%20385%2013485> | markus.seidel at medunigraz.at<mailto:markus.seidel at medunigraz.at> | sent from my mobile phone | please excuse typos and terseness

Am 11.03.2017 um 17:15 schrieb CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>:


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
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