[CIS PIDD] [cis-pidd] EBV+ fulminant infectious mononucleosis/LPD in 2yo girl

[CIS-PIDD]

Dear Elie,

Thanks for your advice, I'll keep you posted on how everything unfolds.

Best,

Arturo

El sábado, 28 de noviembre de 2015, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
escribió:

> Dear Arturo,
> Not sure it is HLH yet, but it looks like an EBV with very severe immune
> reaction. The proliferation is a T-cell proliferation, let's hope it is
> just a reaction to EBV-infected B cells and not a T-cell infection by EBV
> which is rare but very severe.
> Yes, I would begin with Rituximab in order to kill the infected B cells
> that activate the T cells, and I would add steroids (2 mg/kg or a pulse
> depending on the severity of the clinical status). I would not start up
> front with the HLH protocol because a so impressive proliferation does not
> fit with a not so elevated ferritin if it was a HLH context. Of course it
> can be an HLH that begins, but you will see depending on the evolution.
> I suggest you control that PCR EBV has dropped just after the Rituximab in
> order to be sure that the virus was in B cells. You could also do a PCR EBV
> after sorting the cells but not every lab has access to this and Rituximab
> is not dangerous even if T cells are infected. To answer your question, yes
> Rituximab asap.
> I hope it helps.
> All the best
> Elie
>
> Elie Haddad
> CHU Sainte-Justine, Montreal
>
> On Nov 28, 2015, at 04:51, CIS-PIDD <cis-pidd at lists.clinimmsoc.org
> <javascript:;><mailto:cis-pidd at lists.clinimmsoc.org <javascript:;>>>
> wrote:
>
> Dear everyone,
>
> I would appreciate your advice on treatment for the urgent situation of
> this patient.
>
> 2 year-old previously healthy girl of non-consanguineous parents, admitted
> Nov 23 due to 11 days of fever, runny nose and cough, treated at outside
> hospital with clarythromycin to no effect. On admission she was febrile,
> with diffuse lymphadenopathy, hepatosplenomegaly, WBC 20660, Hgb 12.5,
> Platelets 235k, ESR 20, AST 348, ALT 566, LDH 700, Bili 1.99. Extensive ID
> work-up revealed positive EBV PCR in blood. Negative EBV IgG and IgM. All
> other virus and bacteria are negative to date.
>
> Long story short, she has worsened clinically and currently has a WBC of
> 55,320 of which 38,700 are lymphocytes (18% atypical), dropping platelets
> and hemoglobin, dropping transaminases, ferritin 270 (increasing),
> triglicerides 264 (increasing), fibrinogen 189 (dropping).
>
> Lymphocyte populations are as follows:
> CD3+: 34,573, CD4+: 7,193, CD8+: 25,987, CD19: 1,353, NK: 1,546.
>
> We don't have much else of immune work-up results yet because we were
> consulted just yesterday. Lymphnode biopsy done today PM, pending, but
> negative for Gram stain and Ziehl Nielsen.
>
> She appears to have fulminant infectious mononucleosis with EBV-driven
> lymphoproliferative disease and possibly starting HLH. We are considering
> possible ITK or CD27 deficiency.
>
> Any recommendations of treatment and work-up will be very helpful.
> Rituximab ASAP? HLH chemo?
>
> Thanks!
>
> Arturo
>
>
> Dr. Arturo Borzutzky S.
> Profesor Asistente
> Inmunología, Alergia y Reumatología Pediátrica
> Departamento de Enfermedades Infecciosas e Inmunología Pediátrica
> Escuela de Medicina, Pontificia Universidad Católica de Chile
> [
> https://docs.google.com/uc?id=0BwqpIi5-Fh6gUlFpNmZtRWxIQk0&export=download
> ]
> [
> https://docs.google.com/uc?id=0BwqpIi5-Fh6gRGlDcWVwMzlUUUU&export=download
> ]
>
> ---
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