[CIS-PAGID] EBV infection and

[Elie Haddad]

I like the idea of very distal Alemtuzumab
Elie 

Le 2011-12-22 à 11:32, Michael Albert a écrit :


> Eli, since you asked for it:

> One thing is obvious: transplant as soon as possible (from an EBV

> positive donor) as said before.

> The rest is personal opinion: I would treat with ganciclovir, add

> alemtuzumab to the conditioning as it might even bring down EBV load,

> but give it as distal as possible i.e. day-14 or even -21 in order not

> to compromise the donor T-cells too much. This will also help in

> controlling the HLH. I personally would give a reduced intensity

> regimen such as Flu/Mel or maybe Flu/Bu8, because this sounds like a

> sick patient to begin with. Maybe this would be a patient in whom to

> measure alemtuzumab levels on day 0. Cryopreserve T-cells in any case

> and then you might consider a T-cell addback in case there was still a

> considerable alemtuzumab level before transplant. CSA and MMF as GVHD

> prophylaxis and adjust that depending on EBV load and (later)

> chimerism.

> Best,

> Michael

> 

> PD Dr. med. Michael Albert

> Oberarzt

> Abteilung für Pädiatrische Hämatologie/Onkologie

> Leiter der Stammzelltransplantation

> Dr. von Haunersches Kinderspital der LMU

> Lindwurmstr.4

> 80337 München

> Tel: 089 5160 2785

> Fax: 089 5160 4719

> 

> 

> 

> Michael Albert, MD

> Assistant Professor

> Department of Pediatric Hematology/Oncology

> Head SCT Program

> Dr. von Haunersches Kinderspital der LMU

> Lindwurmstr.4

> 80337 München

> Germany

> Tel: +49 89 5160 2811

> Fax: +49 89 5160 4719

> 

> 

> On Thu, Dec 22, 2011 at 3:12 PM, Elie Haddad <elie.haddad at umontreal.ca> wrote:

>> 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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>>