[CIS PIDD] [cis-pidd] CAEBV

Mon May 22 17:58:29 EDT 2017

Thanks Sharat!  I appreciate the invitation.  It would be helpful.  Not urgent - whenever you have time.  Send me a few times / days and I can give you a call.

Dave

dbuchbinder at choc.org

________________________________
From: cis-pidd at lyris.dundee.net <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Monday, May 22, 2017 2:33 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] CAEBV

Dear Dave,

We had a 5 yr old with T-cell CAEBV who was born in China and adopted by a family here in the US. Her EBV quant was 13 million copies at baseline. She had secondary HLH with multi-organ involvement and was quite ill. Genetic testing was negative. She was also enrolled on Jeff Cohen’s genetic study. Her EBV did not respond to a combination of bortezomib/Vorinostat(HDAC inhibitor)/ganciclovir. Her disease responded to alemtuzumab and we used it as a bridge prior to transplant. She proceeded to receive a MUD-BMT following which her disease has been in remission (she is now 18 months post BMT).

Happy to chat offline if needed.

Best,

Sharat

Sharat Chandra, MD

Assistant Professor

Division of Bone Marrow Transplantation and Immune Deficiency

Cincinnati Children's Hospital Medical Center

Cincinnati, OH 45229

Ph. 513 636 5917

Pager: 513 736 0355

From: <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Date: Monday, May 22, 2017 at 4:53 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: Re: [cis-pidd] CAEBV

Thanks Blachy!!!  As we move on...  I would greatly appreciate chatting.  I will keep you number and reach out soon.

Dave Buchbinder

CHOC Children's Hospital

________________________________

From: cis-pidd at lyris.dundee.net <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Monday, May 22, 2017 7:00 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] CAEBV

Dear Dave;

These are tough cases.  Jeff Cohen has a study on genetic studies for CAEBV; you may want to contact him if interested.

Like you mentioned, it will be important to determine what cell line is affected.  We had a patient with T cell SCAEBV, and we treated with bortezomib/ganciclovir.  This did not really dramatically decrease the EBV titers but kept disease under control.   After a while, however, control waned and she had a flare concerning for HLH, so she received stronger lymphoma type therapy to reduce T cell burden (an asparaginase/MTX regimen).

After this, we proceeded to BMT and then gave EBV specific CTL’s post BMT.   You may want to contact Cath Bollard about this.   She is now around 200 days post BMT and doing well with negative EBV titers.

Feel free to contact me offline if you want more details.

-Blachy

Blachy J. Dávila Saldaña, MD

Attending Physician, Division of Blood and Marrow Transplantation

Children's National Health System

Assistant Professor of Pediatrics

George Washington University School of Medicine and Health Sciences

111 Michigan Avenue, N.W.

Washington, DC 20010

Phone: 202-476-4561

Fax: 202-476-2280

From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Monday, May 22, 2017 1:43 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] CAEBV

Dear Colleagues,

I posted a bit ago about a 16 yo male who presented with 3 months fever, cough, weight loss, night sweats,
massive hepatosplenomegaly, cytopenias (mild neutropenia, mild anemia, and moderate thrombocytopenia.  No monocytopenia.  Some hypocellularity and hemophagocytosis.  Fibrinogen low in 60s, now 90s.  Ferritin 3500.  Triglycerides normal. His EBV PCR is persistently elevated (at that time up to around 333,000 copies).  He also had a BAL with EBV isolated by PCR.  No EBV IgM but + EBV IgG VCA and NA.  Immunoglobulins - IgG elevated 1,600 and IgE elevated at 556.   IgM and IgA are normal.  Vaccine responses were lousy.   CD3 was low at 331,  CD4 203, CD8 126. B cells low at 57. NK cells high at 1205.   NK function was normal.  sIL2R was high.  Mitogens fine.  Additional flow based studies for SAP, XIAP, perforin staining --  all negative , etc.  Gene panels for SCID variants, familial forms of HLH, etc -- all negative.  Family hx of a distant male cousin with 3 cancers in 20s, alive (one skin cancer and remainder unknown) but otherwise no males with significant issues (though mostly girls in family).   He disappeared for a bit and was treated by an oncology team with rituximab which helped.  His EBV PCRs dropped.  His platelets got better.  He had a transaminitis that improved.  His organomegaly improved slightly.  His sIL2R normalized, etc.

He is now 17 years old.  He just came to see me because he has low platelets, mod. transaminitis, organomegaly still present, and EBV PCR levels around 2 million copies from his peripheral blood.  His last rituximab dose was in July 2016.  I just sent off a lymphocyte immunophenotyping to see if he has any B cells left.  I am also going to grab a liver biopsy and some clonality studies on his T cells, etc.  I also requested some analysis on his previous marrow exam to look at which cells the EBV is in.  I also requested HLA typing.   At this point he seems to be more of a CAEBV kid rather than a EBV associated HLH.  He is stable and not sick appearing.  If he has B cells...  I can give him some more rituximab to bridge to a unrelated donor transplant using a RIC approach.  If he has no B cells....  I was wondering if anyone would suggest any sort of bridging therapy prior to transplant as it will take a bit of time to secure a donor, etc.   Any comments / suggestions are welcome.

Thanks as always...

Dave Buchbinder

CHOC Children's Hospital

---------------------------------------------------------------------
This email message and any files transmitted are sent with confidentiality in mind and contain privileged or copyright information. You must not present this message to another party without gaining permission from the sender. If you are not the intended recipient you must not copy, distribute or use this email or the information contained in it for any purpose other than to notify Children's Hospital of Orange County. Any views expressed in this message are those of the sender, except where the sender specifically states them to be the views of Children's Hospital of Orange County. If you have received this message in error, please notify the sender immediately, and delete this email from your system. We do not guarantee that this material is free from viruses or any other defects although due care has been taken to minimize the risk.
-------------------------------------------------------------------- ---------------------------------------------------------------------
This email message and any files transmitted are sent with confidentiality in mind and contain privileged or copyright information. You must not present this message to another party without gaining permission from the sender. If you are not the intended recipient you must not copy, distribute or use this email or the information contained in it for any purpose other than to notify Children's Hospital of Orange County. Any views expressed in this message are those of the sender, except where the sender specifically states them to be the views of Children's Hospital of Orange County. If you have received this message in error, please notify the sender immediately, and delete this email from your system. We do not guarantee that this material is free from viruses or any other defects although due care has been taken to minimize the risk.
--------------------------------------------------------------------

---

You are currently subscribed to cis-pidd as: bjdavilas1 at childrensnational.org<mailto:bjdavilas1 at childrensnational.org>.

To unsubscribe click here: http://cts.dundee.net/u?id=101257772.c288ed6a977f295b1281787205c8980d&n=T&l=cis-pidd&o=4386316<https://urldefense.proofpoint.com/v2/url?u=http-3A__cts.dundee.net_u-3Fid-3D101257772.c288ed6a977f295b1281787205c8980d-26n-3DT-26l-3Dcis-2Dpidd-26o-3D4386316&d=DwMFAw&c=Zoipt4Nmcnjorr_6TBHi1A&r=rAv0RMEMpdUphtpo9AaBIUmFcJmqZ-IozxRyfFNABKk&m=WV9m8pTlll1z8Ivs0vFpbSbwc0kExFEbsbD6GdAbr-A&s=kf3ReRKr6wzNo6991IKRiKcW_Rgno6wurgljEoIoSZs&e=>

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-4386316-101257772.c288ed6a977f295b1281787205c8980d at lyris.dundee.net<mailto:leave-4386316-101257772.c288ed6a977f295b1281787205c8980d at lyris.dundee.net>

________________________________

Confidentiality Notice: This e-mail message, including any attachments, is for the sole use of the intended recipient(s) and may contain confidential and privileged information. Any unauthorized review, use, disclosure or distribution is prohibited. If you are not the intended recipient, please contact the sender by reply e-mail and destroy all copies of the original message.

---

You are currently subscribed to cis-pidd as: dbuchbinder at choc.org<mailto:dbuchbinder at choc.org>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396481.905e75689f86143242a7fcc04e69bfcb&n=T&l=cis-pidd&o=4387282<https://urldefense.proofpoint.com/v2/url?u=http-3A__cts.dundee.net_u-3Fid-3D96396481.905e75689f86143242a7fcc04e69bfcb-26n-3DT-26l-3Dcis-2Dpidd-26o-3D4387282&d=DwMF-g&c=P0c35rBvlN7D8BNx7kSJTg&r=3BUsULudXkxI92iImJ0Iy53jCTE37-vi8aHrxdfjhus&m=7d4TpZ9Zb9dE0iXtuBR57e2XkpiZa9Hmcbqnm6yNuZE&s=kEwEmUfXe0VuX0qvRmnYfaMG6lAFAKyXYM4csV9Aoos&e=>

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-4387282-96396481.905e75689f86143242a7fcc04e69bfcb at lyris.dundee.net<mailto:leave-4387282-96396481.905e75689f86143242a7fcc04e69bfcb at lyris.dundee.net>

---------------------------------------------------------------------
This email message and any files transmitted are sent with confidentiality in mind and contain privileged or copyright information. You must not present this message to another party without gaining permission from the sender. If you are not the intended recipient you must not copy, distribute or use this email or the information contained in it for any purpose other than to notify Children's Hospital of Orange County. Any views expressed in this message are those of the sender, except where the sender specifically states them to be the views of Children's Hospital of Orange County. If you have received this message in error, please notify the sender immediately, and delete this email from your system. We do not guarantee that this material is free from viruses or any other defects although due care has been taken to minimize the risk.
-------------------------------------------------------------------- ---------------------------------------------------------------------
This email message and any files transmitted are sent with confidentiality in mind and contain privileged or copyright information. You must not present this message to another party without gaining permission from the sender. If you are not the intended recipient you must not copy, distribute or use this email or the information contained in it for any purpose other than to notify Children's Hospital of Orange County. Any views expressed in this message are those of the sender, except where the sender specifically states them to be the views of Children's Hospital of Orange County. If you have received this message in error, please notify the sender immediately, and delete this email from your system. We do not guarantee that this material is free from viruses or any other defects although due care has been taken to minimize the risk.
--------------------------------------------------------------------

---

You are currently subscribed to cis-pidd as: sharat.chandra at cchmc.org<mailto:sharat.chandra at cchmc.org>.

To unsubscribe click here: http://cts.dundee.net/u?id=97587726.e970f7092c6a4089ef5dc1c8b70ae1f3&n=T&l=cis-pidd&o=4389234<https://urldefense.proofpoint.com/v2/url?u=http-3A__cts.dundee.net_u-3Fid-3D97587726.e970f7092c6a4089ef5dc1c8b70ae1f3-26n-3DT-26l-3Dcis-2Dpidd-26o-3D4389234&d=DwMF-g&c=P0c35rBvlN7D8BNx7kSJTg&r=3BUsULudXkxI92iImJ0Iy53jCTE37-vi8aHrxdfjhus&m=7d4TpZ9Zb9dE0iXtuBR57e2XkpiZa9Hmcbqnm6yNuZE&s=P6PLI7LU2pWfaYqzhP2Ilx9-kekmg3qUnaqjWILxPiA&e=>

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-4389234-97587726.e970f7092c6a4089ef5dc1c8b70ae1f3 at lyris.dundee.net<mailto:leave-4389234-97587726.e970f7092c6a4089ef5dc1c8b70ae1f3 at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: dbuchbinder at choc.org<mailto:dbuchbinder at choc.org>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396481.905e75689f86143242a7fcc04e69bfcb&n=T&l=cis-pidd&o=4389285

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-4389285-96396481.905e75689f86143242a7fcc04e69bfcb at lyris.dundee.net<mailto:leave-4389285-96396481.905e75689f86143242a7fcc04e69bfcb at lyris.dundee.net>

---------------------------------------------------------------------
This email message and any files transmitted are sent with confidentiality in mind and contain privileged or copyright information. You must not present this message to another party without gaining permission from the sender. If you are not the intended recipient you must not copy, distribute or use this email or the information contained in it for any purpose other than to notify Children's Hospital of Orange County. Any views expressed in this message are those of the sender, except where the sender specifically states them to be the views of Children's Hospital of Orange County. If you have received this message in error, please notify the sender immediately, and delete this email from your system. We do not guarantee that this material is free from viruses or any other defects although due care has been taken to minimize the risk.
-------------------------------------------------------------------- ---------------------------------------------------------------------
This email message and any files transmitted are sent with confidentiality in mind and contain privileged or copyright information. You must not present this message to another party without gaining permission from the sender. If you are not the intended recipient you must not copy, distribute or use this email or the information contained in it for any purpose other than to notify Children's Hospital of Orange County. Any views expressed in this message are those of the sender, except where the sender specifically states them to be the views of Children's Hospital of Orange County. If you have received this message in error, please notify the sender immediately, and delete this email from your system. We do not guarantee that this material is free from viruses or any other defects although due care has been taken to minimize the risk.
--------------------------------------------------------------------

---
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=4389312
or send a blank email to leave-4389312-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20170522/16254755/attachment-0001.html>