[CIS PIDD] [cis-pidd] infant with aseptic meningitis, ITP, fever, and hepatosplenomegaly

[CIS-PIDD]

Dear Jordan,
Agree with Markus. Sometimes JMML is unravelled by acute CMV infection in
the early postnatal period.
ATB
Nacho


*Luis I. Gonzalez-Granado. MD.*Immunodeficiencies Unit. Hospital 12 de
octubre.Research Institute Hospital 12 octubre (i+12)
Av. Cordoba S/N. 28041. Madrid. Spain
Tel. *0034**606732959 / * 0034913908569  /  Fax 0034913908772
<0034934893039>luisignacio.gonzalez at salud.madrid.org
<luisignacio.hdoc at salud.madrid.org>ORCID ID:  orcid.org/0000-0001-6917-8980
Researcher ID: B-9257-2009ResearchGate:
https://www.researchgate.net/profile/Luis_Gonzalez-Granado LinkedIn:
https://es.linkedin.com/in/nachgonzalez

2017-04-20 7:13 GMT+02:00 CIS-PIDD <cis-pidd at lists.clinimmsoc.org>:

> Dear all,
>
> We were called to consult on a now 3-month-old girl with aseptic
> meningitis and ITP. She had an unremarkable birth history. She initially
> presented at 10 week old with persistent fever despite being well
> appearing. After being sent away from 2 ERs, the third ER performed a
> lumbar puncture with 1000 WBC and no red cells. There was an even spread
> between PMN, monos, and lymphocytes. Protein mildly elevated and glucose
> mildly reduced. Bacterial, fungal, mycobacterial, and viral studies have
> been negative from CSF and blood. Over the past 3 weeks, LP has been
> repeated twice with similar result. Fevers have been intermittent despite
> appropriate antibiotic therapy, and clinically she appears well. MRI of the
> brain and spine have demonstrated leptomeningeal enhancement extending to
> cervical nerve roots consistent with the CSF findings. There was restricted
> diffusion in the extra-axial space over the temporal lobes. No basal
> ganglia calcification identified on head CT or MRI.
>
> During her admission for the above, she was noted to have mild
> hepatosplenomegaly by abdominal ultrasound, and then she subsequently
> developed immune thrombocytopenia with confirmed platelet glycoprotein
> antibodies detected in her blood. Her platelet count was normal at the time
> of admission. No other organ disease has been identified. There is no rash,
> and she has normal growth with normal PO intake and no evidence of
> enteropathy. She has had temps as high as 101.5 on 3 occasions, and outside
> of fever, her clinical appearance unremarkable.
>
> Initial immune studies have been sent and are copied below:
> WBC 23200 (ANC 4500, AMC2500, ALC 20000, AEC1100)
> CD19 6475 (44.3%)
> CD8 1768 (12.1%)
> CD4 5320 (36.4%)
> CD3 7118 (48.7%)
> CD16/56 804 (5.5%)
> CD45RO 11.3
> CD45RA 88.1
>
> IgM (164)
> IgG (689)
> IgA <40.
> IgE 5
>
>
> Bone Marrow showed monocytosis with normal cellularity (reported as 100%)
> and trilineage hematopoiesis.  No excess blasts or hemophagocytes. Normal
> cytogenetics.  Additional genetic studies for JMML are pending.
>
> We are arranging for trio exome sequencing.
>
> Questions:
> 1. Does the presence of ITP rule out an infectious cause for the
> meningitis?
> 2. What additional workup would you recommend to assist in diagnosis (much
> workup was left out for the sake of brevity)?
> 3. Has anyone been in the same or similar situation?
> 4. The marked elevation of all white blood cell lineages suggests
> widespread dysregulation. We are considering starting corticosteroids. Is
> there any reason to not do so or to try a different immunosuppressive
> medication?
>
> Thanks in advance for your thoughtful responses!
>
> Jordan
>
> Jordan Abbott, MD
> National Jewish Health
>
>
>
>
>
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