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

[CIS-PIDD]

Thanks, James! I really hope that does not happen to this patient. I will discuss all of this with the team.

________________________________
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 10:56 AM
To: CIS-PIDD
Subject: RE:[cis-pidd] infant with aseptic meningitis, ITP, fever, and hepatosplenomegaly

I agree with all of the comments.

One thought is that if you think this is nomid/NLRP3 sometimes its easier to just try anakinra and see if there is a dramatic response.  Several days of anakinra is unlikely to hurt the patient and personally I feel its safer than steroids….although I wouldn’t be against trying steroids also

If you figure this out let me know since I had a very similar patient who progressed to liver failure and passed away…we sequenced but did not find a likely culprit.  Had started with aseptic meningitis that looked bacterial but no positive cultures and did not respond to antibiotics, thrombocytopenia developed (we didn’t check Abs), then hepatitis, coagulopathy.  Tried anakinra, steroids, and even etoposide and dex for presumed HLH but nothing helped.

Best

James


James W. Verbsky M.D./Ph.D.
Associate Professor of Pediatrics and Microbiology
Medical Director, Clinical Immunology Research Laboratory
Medical Director, Clinical and Translational Research
Medical College of Wisconsin
Milwaukee, WI



From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Thursday, April 20, 2017 12:13 AM
To: CIS-PIDD
Subject: [cis-pidd] infant with aseptic meningitis, ITP, fever, and hepatosplenomegaly

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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