[CIS PIDD] [cis-pidd] Splenic embolization in CVID with massive splenomegaly

[CIS-PIDD]

Dear colleagues,


I am caring for a 50 yo male with 20 years+ history of CVID and massive splenomegaly, portal hypertension with complications of esophageal varices. I was wondering if anyone has experience with debulking spleen through a staged embolization in CVID cases.


Patient now has nearly absent IgGAM, absent vaccine responses, pancytopenia (markedly low ANC, ALC, platelets), GLILD s/p rituximab, massive splenomegaly and ascites. He recently suffered from SBP. There are small granulomas in the liver although do not appear to be significant enough to cause non-cirrhotic portal hypertension. Recent liver biopsy did not show obvious cirrhosis, although there could be sampling error. The imaging was reviewed with radiology and his GI believes that the massive spleen may be drawing significant inflow and resulting significant outflow into the portal vein despite a normal liver. Although they do not treat portal hypertension this way, the GI team thinks he might benefit from debulking of his spleen through a staged embolization so he would still have remaining spleen.


WES has been sent and is pending. We are considering getting a BM biopsy and evaluation for BMT.


I would appreciate any advice regarding treatment of massive splenomegaly resulting in portal hypertension in CVID cases.


Thank you,

Panida


Panida Sriaroon, MD
Associate Professor
Director, Fellowship Program
Medical Director, USF/All Children's Hospital Allergy/Immunology clinic
Beeper 727.825.4379
Office 727.553.3521
E-mail:psriaroo at health.usf.edu

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=4291726
or send a blank email to leave-4291726-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20170329/30a14961/attachment-0001.html>