[CIS-PAGID] Puzzling Patient #1

[Seppänen Mikko]

Never ever seen such,

however, the differential in the literature of necrotizing cervical adenitis, as I remember it, is VERY short, usually if no bacterial pathogens are isolated, only IV drug use (any history of that?), Kikuchi-Fujimoto and lupus (lupus serology?) are mentioned (or was there an occasional Castleman as well?).

However, since I have a what I have called CVIDs-patient who presented with this (and necrotizing adenitis was thought to have been caused by IVDU), I guess I need to check... I'll tell if it comes out positive.... But Yours has no CVIDs.

mikko

Helsinki, Finland

Lähettäjä: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] Puolesta Church, Joseph
Lähetetty: 3. toukokuuta 2012 5:02
Vastaanottaja: pagid at list.clinimmsoc.org
Aihe: Re: [CIS-PAGID] Puzzling Patient #1

None.  JC

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Richard Wasserman
Sent: Wednesday, May 02, 2012 6:53 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Puzzling Patient #1

Any other history of infection?
Richard Wasserman
Dallas
On Wed, May 2, 2012 at 8:47 PM, Church, Joseph <JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>> wrote:
This 18yo girl had persistent anterior cervical adenitis complicating severe purulent tonsillitis.  Throat culture was negative for bacterial pathogens.  Routine serology for EBV and CMV were negative.  There was no organomegaly or adenopathy other than that described.

Eventually she came to biopsy that showed necrotizing adenitis with strong histochemical staining for EBV.  Histologic and histochemical features were not consistent with neoplasm.

EBV serology 1 month after the procedure showed only +EBV VCA IgM; 3 months after the procedure the serologic picture was the same.

Blood PCR for EBV was negative x2.

The following immune functions were normal:
            T-, B-, NK cell numbers.
            Ig levels
            Antibody responses to Pneumovax
            Mitogen and antigen-specific LPAs
            CTL function (Cincinnati Children's Lab)
            NK cell function (Cincinnati Cildren's Lab)
            X-inactivation - random (looking for potential for XLP)

Have any of you seen 4+ EBV in a lymph node with negative VCA IgG and EBNA and negative blood EBV PCR?

What am I missing?

Joe Church
Children's Hospital Los Angeles

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DallasAllergyImmunology
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