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

Thu Apr 20 09:29:47 EDT 2017

around 30% PMN, 30% lymphocyte, 30% monos/macrophages
________________________________
From: cis-pidd at lyris.dundee.net [cis-pidd at lyris.dundee.net] on behalf of CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, April 20, 2017 4:48 AM
To: CIS-PIDD
Subject: Re: [MARKETING] Re: [cis-pidd] infant with aseptic meningitis, ITP, fever, and hepatosplenomegaly

The WBC on VHS puncture is neutrophils or lymphocytes?

Sent from my iPhone

On 20 Apr 2017, at 05:43, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

I would rule FHL and autoinflammatory diseases out. We had a similar case due to perforin deficiency (no hemophagocytosis in BM at the beginning)

Best,

Pere Soler Palacín, MD, PhD, MSc.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron.
Vall d'Hebron Research Institute (VHIR)
Assistant Professor. Universitat Autònoma de Barcelona (UAB)
Director of the Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies (Barcelona)
Pg. de la Vall d'Hebron, 119-129
08035 Barcelona. Spain.
Tel. 0034934893140  /  Fax 0034934893039
psoler at vhebron.net<mailto:psoler at vhebron.net>  /  34660psp at comb.cat<mailto:34660psp at comb.cat>
Web: www.upiip.com<http://www.upiip.com>
My ORCID
ResearchGate: http://www.researchgate.net/profile/Pere_Soler-Palacin
LinkedIn: www.linkedin.com/in/pere-soler-palacin<http://www.linkedin.com/in/pere-soler-palacin>

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________________________________
De: "PAGID" <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Per: "CIS-PIDD" <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Enviats: Dijous, 20 de Abril 2017 8:29:27
Assumpte: [MARKETING] Re: [cis-pidd] infant with aseptic meningitis, ITP, fever, and hepatosplenomegaly

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. 0034606732959 /  0034913908569  /  Fax 0034913908772<tel:0034934893039>
luisignacio.gonzalez at salud.madrid.org<mailto:luisignacio.hdoc at salud.madrid.org>
ORCID ID:  orcid.org/0000-0001-6917-8980<http://orcid.org/0000-0001-6917-8980>
Researcher ID: B-9257-2009
ResearchGate: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<mailto: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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