[CIS-PAGID] EBV infection and

[Elie Haddad]

Given the absent cytotoxicity and the absent degranulation, it is very likely that this patient has a "true" primitive HLH and you should find mutation in Munc13-4, Munc18-2 (XLP related genes should be excluded since she is a girl) Also, look for partial albinism with hair microscope analysis. Therefore, transplantation is absolutely required (even if you don't find mutation).
Since you observe so many copies afer Rituximab and you don't find circulating B cells, then EBV in in T lymphocytes or NK cells.
I do agree with Jeff Cohen for transplantation: the sooner the better. To reduce EBV load before BMT, I don't see other means than Ganciclovir.
In patients with chronic EBV infection who have been transplanted, some have been described with good evolution without engraftment suggesting that the conditionning regimen has "destroyed" the infected T or NK cells.
The tough question is about GvHD prophylaxis. since it is a MUD, one ould like to be strong against GvHD, but since you will like a rapid immune reconstitution to control EBV, one would like to be weak..... Therefore, one could say no ATG and no alemtuzumab, but it is also possible that ATG or Alemtuzumab will induce a deep decrease of EBV load....
I would go with a "classic" BMT with Bu-Flu (myeloablative) and CsA-MTX, but as I said, a reduced CR with serotherapy for GvH prophylaxis could be also a good option.
I would like to hear the opinion of other "transplanters" in this case.
Elie Haddad 

Elie Haddad, MD, PhD,
Professor of Pediatrics, University of Montreal,
Head, Pediatric Immunology and Rheumatology Division,
CHU Sainte-Justine, 3175 Cote Sainte-Catherine
Montreal, QC, H3T 1C5, Canada
Ph: 1 514 345 4713
fax: 1 514 345 4897
e-mail: elie.haddad at umontreal.ca





Le 2011-12-22 à 03:05, Pere Soler Palacin a écrit :


>  

> 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; 34660psp at comb.cat. Web: www.upiip.com.

> 

>  

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