[CIS PIDD] [cis-pidd] CAEBV

[CIS-PIDD]

Dear David,
tough case, while you are in the way of HSCT, CTL'S may help. We tried it
in the past in one patient with some success.Of course the sooner  you can
transplant him,  the better.
All the best
Nacho

El lun., 22 may. 2017 7:43, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
escribió:

> 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
>
>
>
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