[CIS-PAGID] Puzzling Patient #2

[Church, Joseph]

Thanks, Mikko.

Kikuchi syndrome is in the differential;  EBV serology and PCR were negative.  ANA is positive at 1:320.  He does not have dry eyes or dry mouth.

Our rheumatologists started him on steroids, and he had a fairly impressive reduction in fever pattern and parotid swelling after only 2 doses.

JC




-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Seppänen Mikko
Sent: Wednesday, May 02, 2012 10:32 PM
To: 'pagid at list.clinimmsoc.org'
Subject: Re: [CIS-PAGID] Puzzling Patient #2

Agree with Donald about the serology pitfalls, fully.  Could be EBV-HLH?

I would also check for lupus (see discussion on Pt#1), which can cause both necrotizing adenitis and autoimmune parotitis. Does the patient have dry eyes?

mikko

Helsinki
FINLAND



-----Alkuperäinen viesti-----
Lähettäjä: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] Puolesta Donald Cuong Vinh, Dr
Lähetetty: 3. toukokuuta 2012 5:55
Vastaanottaja: pagid at list.clinimmsoc.org
Aihe: Re: [CIS-PAGID] Puzzling Patient #2

The mumps IgM may be a false-positive result, while the IgG could be from the previous vaccination.

There are a few viral infections that can cause false-positive mumps IgM (Davidkin et al., J Infect Dis 2005). Of note, EBV infection is one of them (heterophile antibodies detected in monospot).

If you are convinced that this is clinically mumps in this previously vaccinated (x2) child, you may have to do alternate testing (e.g. buccal/oral swab and concomitant blood sample sent to a reference lab).

Donald C. Vinh, MD
McGill University Health Centre - Montreal General Hospital
1650 Cedar Ave, Rm A5-156
Montreal, Quebec,  Canada H3G 1A4



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________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] on behalf of Church, Joseph [JChurch at chla.usc.edu]
Sent: Wednesday, May 02, 2012 10:01 PM
To: pagid at list.clinimmsoc.org
Subject: [CIS-PAGID] Puzzling Patient #2

I was just asked to consult on a 9yo boy with a 1 month history of progressive bilateral parotitis associated with spiking fevers.  Multiple features of his case suggested macrophage activation syndrome (MAS) although he did not have organomegaly.  Bone marrow showed hypocellularity with no evidence of malignancy.  Lymph node biopsy showed non-granulomatous necrotizing adenitis with preserved T and B cell populations, and lots of macrophages.

Serologic studies demonstrated strongly positive IgG and IgM against mumps virus.

His past medical records document that he was fully immunized (x2) with MMR.

Has anyone seen

·         mumps virus-associated MAS?

·         familial hemophagocytic lymphohistiocytosis presenting with mumps?

Why did this kid get mumps?

Joe Church
Children's Hospital Los Angeles



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