[CIS-PAGID] EBV infection and

[Fleisher, Thomas (NIH/CC/DLM) [E]]

I passed your email on the our EBV expert Dr. Jeff Cohen and his response is below:

"The sooner she can be transplanted and have a healthy immune system, the better.

One might also look for evidence of EBV lymphoproliferative disease by CT in this patient.

One thing that could be tried (I don't have much experience with it) is ganciclovir.  If there is active virus lytic replication (not replication of just latently infected cells), then ganciclovir would become phosphorylated in the virus infected cells undergoing lytic replication, which would kill the cells.   If a lab could test for encapsidated EBV DNA in the plasma (EBV DNA inside viral nucleocapsids that is resistant to DNAse, unless the plasma is treated with proteinase K to release the EBV DNA) that would indicate that there is lytic viral DNA replication.  If instead, this is due to proliferation of only latently infected cells, then ganciclovir would not be effective and would only have toxicity.  I am not impressed with the use of foscarnet in this setting for EBV and would not use it for treatment of EBV.

If ganciclovir is tried let me know, as I hear about patients like this and would be interested to know if it has an effect in this type of setting."

If you would like to communicate directly with him, please send me an email and I will connect you.
Tom

Thomas A. Fleisher, M.D.
Chief, Department of Laboratory Medicine
NIH Clinical Center
301 496-5668 (T)
301 402-1612 (F)
From: Pere Soler Palacin [mailto:psoler at vhebron.net]
Sent: Thursday, December 22, 2011 3:05 AM
To: PAGID
Cc: lalsina at hsjdbcn.org
Subject: [CIS-PAGID] EBV infection and




Dear colleagues, this patient is currently being treated in another centre, here in Barcelona. We would appreciate your opinion about this case.



Girl, 23 months-old, diagnosed in June 2011 of FHLH (EBV-induced). Absent cytotoxicity, normal perforin expression, absent degranulation, pending results of genetic studies. No CNS involvment. Treated following HLH-2004 protocol, with good response to induction treatment (8 weeks) followed by maintenance treatment. Has had 2 relapses related to intercurrent infections, again well controlled with HLH2004 treatment intensification. She is pending a MUD (10/10) which is planned in one month aproximately. Unfortunately, she is currently with an uncontrolled EBV infection (>80 million copies/mm3 in plasma, ferritin 40000 ug/L), despite 5 doses of Rituximab (has <10 B cells/mm3), and treatment with acyclovir, followed by foscarnet,. There are no signs of active hemophagocytosis (6% of CD8+DR+ cells), under full HLH-2004 treatment: DXM 10mg/m2 daily + VP16 twice weekly + cyclosporine.

What would you suggest regarding EBV treatment before BMT?

Thank you in advance,

Pere

Pere Soler Palacín, MD, PhD.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron.
Assistant Professor. Universitat Autònoma de Barcelona.
Passeig de la Vall d'Hebron 119-129.
08035 Barcelona. Spain.
Tel: 0034934893140. Fax: 0034934893039.
E-mail: psoler at vhebron.net<mailto:psoler at vhebron.net>; 34660psp at comb.cat<mailto:34660psp at comb.cat>. Web: www.upiip.com<http://www.upiip.com/>.



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