[CIS PIDD] [MARKETING][cis-pidd] Persistent EBV?

[CIS-PIDD]

Hi Jason,

Agree w Kate´s advice. The only PIDs that spring to mind as  possibilities, would be a mildly symptomatic PIK3CD or late-onset GATA2.

Thinking of PIK3CD, one would mostly expect to see lymphadenopathy even chronically, and at least when the patient is symptomatic (and in mono like illness that would be the case). One could take a LN biopsy and immunostain for EBV (+blood EBV and CMV PCR again), and if positive, I would consider testing for that specifically. Being AD and at times di novo, it would have repercussions for follow up, expected complications, genetic counselling, testing relatives a.s.f. I would BTW also screen for HLH, when next time symptomatic.

And even if monocytes are normal, I would love to see that B- (CD4, CD8) and NK cells are as well (thinking of GATA2) and ask about familial leukemia or like. Not all adults even have low monocytes when they become symptomatic, but all I have seen have had low B and NK cells.

Serology in itself does not worry me, but those episodes do to an extent. And yes, SLE seems like a possibility?

Mikko

Mikko Seppänen, Finland

Lähettäjä: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Lähetetty: 2. lokakuuta 2015 21:12
Vastaanottaja: CIS-PIDD
Aihe: [MARKETING][cis-pidd] Persistent EBV?


I would appreciate any comments regarding the patient below.  My questions are as follows:

  1.   Clinical significance of several years or more of persistently elevated EBV titers and one positive (although < 100 copies/mL) PCR during an asymptomatic period?
  2.  To what extent does this patient need additional immunologic evaluation?
  3.  To what extent does this patient need treatment (if at all)?

53 year-old woman who recounts 4 episodes of 'mono-like' illness since her early 20s.

Typical presentation is fever, pharyngitis, cervical lymphadenopathy and fatigue; no rash.  I cannot find documentation of splenomegaly. Each time, EBV serology revealed elevated VCA IgG and IgM; elevated EBNA IgG; elevated EA IgG.  She has NOT had EBV PCR performed during these episodes.  She has not had elevated LFTs or cytopenias.   It takes 3-4 weeks or more for her to recover.  She MAY have had convalescent serology noting negative VCA IgM and EA on one occasion, although I cannot confirm this.

She was started on valacyclovir through primary care several months before presentation to our clinic; reason for treatment and rationale for dose uncertain.

Presently (5 months after her most recent episode, asymptomatic), EBV VCA Ig and IgM are elevated, as are EBNA IgG and EA IgG (similar to previous).  EBV quantitative, whole blood (Lab Corp) PCR- while on valacyclovir 500mg once daily - was 'positive' but at < 100 copies/mL.  I confirmed this with lab tech.  It has not been repeated.

Health history includes recurrent upper respiratory illness in the context of atopy (allergic rhinitis, EIA) and IgE-mediated anaphylaxis to tree nuts.

Between episodes she has NO history of [relapsing-remitting] fever, lymphadenopathy, hepatosplenomegaly or rash; no elevation of liver enzymes; no cytopenias.  She has NOT had invasive bacterial or opportunistic infections.  She has had NO history of malignancy, autoimmunity, interstitial lung disease or CNS disease.

She is adopted; family history is unavailable.

CBC, CMP, quantitative immunoglobulins, post-vaccine tetanus/pneumococcal titers, TBNK cell enumeration, mannose-binding lectin are NORMAL/within reference range/unrevealing.  NO OTHER DIAGNOSTICS HAVE BEEN PERFORMED.

So my questions are:

  1.  In the context of this patient's history, what to make of her persistently elevated EBV serology and single finding of positive (although < 100 copies/mL) PCR during an asymptomatic period (while on valacyclovir)?
  2.  To what extent does this patient need additional immunologic evaluation?
  3.  To what extent does this patient need treatment (if at all)?



Thank you for your thoughts.



-j

Jason Raasch, MD

Midwest Immunology Clinic
15700 37th Ave N, Ste 110
Plymouth, MN 55446

TEL: (763) 577-0008
FAX: (763) 577-0192







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