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

[CIS-PIDD]

Hi Panida,

A few questions regarding the case: Is the lymphopenia only B cell lymphopenia or are other cells depressed as well? Is there skewing within the T cell repertoire? Was GLILD treated with other immunosuppression other than rituximab and is he still on any? Is there any evidence of enteropathy? Is there any other evidence of lymphoproliferative disease (enlarged lymph nodes, clonality in the serum, clonality in the marrow)? If the latter is true, I might consider the addition of sirolimus before totally taking out the spleen. If there is no obvious evidence, you may need to go looking.

Happy to discuss more,

Jen Leiding

From: "cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>"
Reply-To: "cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>"
Date: Tuesday, March 28, 2017 at 10:04 PM
To: "cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>"
Subject: [cis-pidd] Splenic embolization in CVID with massive splenomegaly


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<mailto:psriaroo at health.usf.edu>

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