[CIS PIDD] [cis-pidd] XLA and angioedema & sub-q IgG

[Mel.Berger at cslbehring.com]

This may be an interesting example of allergy in Bruton's, as suggested by its relation to the introduction of the dogs. I believe I have seen reports of basophil activation by antibiotics in Bruton's patients who are "allergic".

Mel Berger

Sent from my iPhone

On May 12, 2015, at 13:43, Cooper, Megan <Cooper_M at kids.wustl.edu<mailto:Cooper_M at kids.wustl.edu>> wrote:

Hi everyone,

I’m wondering if people have seen recurrent angioedema in their XLA patients?

I treat 2 brothers, age 5 & 2 with XLA who for the past 6 months have both have recurrent episodes of eyelid/periorbital swelling.  These were initially treated as infection, but with recurrence have resolved after 2-3d without antibiotics.  There are no other associated symptoms, although some have occurred concurrent with URI symptoms.  C4 is normal during the episodes and C1 esterase activity is normal.

These episodes started shortly after changing from IV to sub-q IgG (weekly Hizentra), so that is an obvious concern.  Our fellow found something about angioedema on the manufacturer website, but I couldn’t find other evidence and mom is very hesitant to switch back to IV infusions for these young boys.  Dogs were also introduced into the household around that time and for whatever it is worth in these pts skin testing was negative.  High dose antihistamines have had minimal impact.

Thanks,

Megan


Megan A. Cooper, MD, PhD
Assistant Professor, Department of Pediatrics
Division of Rheumatology
Washington University School of Medicine
Cooper_m at kids.wustl.edu<mailto:Cooper_m at kids.wustl.edu>
Lab website: http://research.peds.wustl.edu/Default.aspx?alias=research.peds.wustl.edu/Labs/Cooper_M
(lab office) 314-286-0262
(lab fax) 314-286-2895




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