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

[CIS-PIDD]

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

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