[CIS PIDD] [BULK] Re:[cis-pidd] EBV associated PID

Tue May 24 15:00:08 EDT 2016

Thanks Mike.  I appreciate the recs.

Dave

>>> "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> 5/24/2016 11:55 AM >>>
Hi Dave, 

This sounds extremely concerning for potential CAEBV with EBV-driven NK/T cell lymphoma.

We have a few of these kids that are being treated and worked up for HSCT.   I would highly recommend that you contact Jeff Cohen at NIH to arrange for flow-sorted EBV testing to see which lineage is infected.    If it's not in B-cells, the usual treatment is ganciclovir/velcade to try get the viral count under control, followed by HSCT.  

Our division chief (Catherine Bollard) has treated a number of these patients with EBV-CTLs post-HSCT, and had fairly good outcomes. 

Best,
Mike

Michael D. Keller, MD
Assistant Professor, Division of Allergy / Immunology
Center for Cancer and Immunology Research
Jeffrey Modell Diagnostic and Research Center for 
Primary Immunodeficiency Disorders
Children's National Health System
111 Michigan Ave NW, M7745A
Washington, DC 20010
Clinic: 202.476.3016

Office: 202.476.5843
Fax: 202.476.2280
www.ChildrensNational.org

From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, May 24, 2016 2:36 PM
To: CIS-PIDD
Subject: RE: [BULK] Re:[cis-pidd] EBV associated PID

Anti-EBNA?

Tony Infante

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org] 
Sent: Tuesday, May 24, 2016 1:17 PM
To: CIS-PIDD
Subject: [BULK] Re:[cis-pidd] EBV associated PID

Dear Colleagues,

We have a 16 yo male who presented with 3 months fever, cough, weight loss, night sweats, 
massive hepatosplenomegaly, cytopenias (mild neutropenia, mild anemia, and moderate thrombocytopenia.  No monocytopenia.  Some hypocellularity and hemophagocytosis.  Fibrinogen low in 60s, now 90s.  Ferritin 3500, now 1000s.  Triglycerides normal. His EBV PCR is persistently elevated (now 333,000) - one month ago it was in 45,000 range.  No EBV IgM but + EBV IgG.  Immunoglobulins - IgG elevated 1,600 and IgE elevated at 556.   IgM and IgA are normal.  Vaccine responses are pending.  CD3 low at 331,  CD4 203, CD8 126. B cells low at 57. NK cells high at 1205.   NK function is normal.  Mitogens are pending.  Additional flow based studies for SAP, XIAP, perforin staining, etc. - are pending.  A gene panel for SCID variants was also sent.  Family hx of a distant male cousin with 3 cancers in 20s, alive (one skin cancer and remainder unknown) but otherwise no males with significant issues (though mostly girls in family).   We planning on treating with rituximab to assist with EBV control with avoidance of dex and etoposide since he is clinically stable.  HLA typing has also been requested.  Any thoughts are welcome with respect to potential diagnostic considerations for sequencing in the realm of combined immunodeficiency and / or therapy.

Thanks as always,

Dave

Dave Buchbinder, MD

CHOC Children's Hospital

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