[PAGID] from thread April 10th

[Paige wickner]

 Dear PAGID members- Thank you so much for your assistance and thoughtful
input on this case.

 To answer the questions posted so far:

1. Age of CVID diagnosis:

KC (DOB:05/25/93) was diagnosed with selective IgG2 deficiency in his second
year of life at a different institution.



The diagnosis of CVID was made more recently along with the observation of
declining cellular component.



2. Your patient, again, illustrates how useful CD45RA/RO is in the workup.
Was this done previously (i.e. something to compare to)?



His flow cytometry in 2007 showed:

CD4+CD45RA+ 15%, 177 (ref 241-846)

CD4+CD45RO+ 36%, 425 (ref 246-757)


Flow in 2009 showed:

CD4+CD45RA+ <1%

CD4+CD45RO+46%, 427

Certainly there is a decline on his CD4+naïve T cell population over time.
Sequestration at sites of active infections could accentuate its deficit in
PBLs enumeration.



 3. What about viral PCRs in blood and bone marrow (CMV, EBV, HHV-6, Parvo,
etc)?

 This has not been done, although we will discuss this with the
hematopathologist.



 4. Does he have an abnormal facies or any other characteristic phenotype,
to think about ICL or other DNA repair defect (AT, Nijmegen) that usually
present a progressive disease?

No, KC is of no syndromic phenotype but rather normal, healthy, athletic
appearance.



5. ADA deficiency

We do plan to test him for ADA deficiency. He did have PRBC transfusion
during this admission so we will be looking at ADA levels in other cell
lines.

Recent clinical developments: KC had a good improvement in his oral lesions
over the weekend with resolution of all visible skin/mouth lesions and was
able to once again tolerate PO.  He was discharged Sunday to home on
prophylactic Bactrim, Valcyclovir, Fluconazole, IVIG/monthly, and will be
returning to our clinic this Thursday for follow up. Pending his PBLs count,
we also plan to repeat his proliferation and flow at this time.

I will keep you posted.

Thank you.

Sincerely,

Paige Wickner
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