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

[CIS-PIDD]

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<mailto:cis-pidd at lists.clinimmsoc.org>> 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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