[CIS PIDD] [cis-pidd] reactions to SCIG

[Nacho Gonzalez]

Dear Richard,

Two suggestions:
1) Push therapy (1g) with daily infusion
2) May be worth a try of subcutaneous desensitization? There is a
report from Montreal with success, reported in CIS meeting 2008 (the
patient came from IVIG but still may work to tolerate a higher amount
of SCIG):
" In 1995, a 34-year old woman with CVID developed glottic edema, stridor,
dyspnea and hypotension secondary to infusion of IVIG. The patient was
IgA-deficient but lacked Anti-IgA antibodies. IVIG treatment was
stopped for 10 years due to a risk of anaphylaxis. Because of
recurrent sino-pulmonary and gum
infections difficult to control and antibiotic allergies, she was
given a different commercial IVIG in a graded fashion. She tolerated
this treatment on a monthly basis with premedication (hydrocortisone
and acetaminophen). In 2007, she missed 4 months of therapy, when IVIG
was re-introduced. She developed generalized urticaria, facial
angioedema, throat tightness and shortness of breath and IVIG was
permanently discontinued. A month later, she was hospitalized for a
one-day desensitization protocol with subcutaneous (s.c.)
immunoglobulin. Graded quantities (0.1, 0.5, 1.0, 2.0 and 5.0 cc) of
0.16g/cc s.c. injections were given
every 30 min. Vital signs were monitored pre and post injections.
Spirometry was also carried out before every injection. The patient
had an asymptomatic transient
hypotension after 0.1 cc of subcutaneous immunoglobulin. The following
day, a full dose (10cc) was given in a single s.c. injection and
maintained on a daily basis. No acute or delayed reactions occurred.
She has now tolerated 5 weeks of
treatment without any pre-medication and has maintained a serum level
of IgG of 9.74 g/L" doi:10.1016/j.clim.2008.03.186

Best regards,

Luis Ignacio Gonzalez-Granado
Immunodeficiencies & Pediatric Infectious Diseases Unit
Hematology & Oncology Unit.
Hospital 12 octubre.
Madrid. Spain

2014-06-21 14:09 GMT+02:00 Richard Wasserman <drrichwasserman at gmail.com>:
> Colleagues,
>
> I am posting this patient for a colleague.
>
> She is a 49 yo with a history of sinopulmonary infections but no evidence of
> bronchiectasis. Her IgG ranges from 250 to 350/dL, her IgM is normal her IgA
> is in the 40s. She has never been treated with IGIV products.
>
>
>
> She tolerated the first Hyzentra infusion without a problem and the second
> one (given three weeks later) was completed but she then developed itching,
> tongue swelling and wheezing 4 hours after the completion of the infusion.
> She was treated in the emergency department but did not receive epinephrine.
>
>
>
> Prior to the second infusion ACE inhibitor treatment was begun. She had
> similar reactions around that time to inactivated influenza vaccine and an
> antibiotic. The ACE inhibitor was discontinued.
>
>
>
> A few weeks later, she was treated with Gamunex-C (15g subq). Again, there
> were no problems during the infusion but she developed itching, wheezing and
> tongue swelling several hours later.
>
>
>
> Several weeks later she was given 1g of Gammagard subq and within minutes
> she developed generalized and tongue pruritis and wheezing.
>
>
>
> Another Gammagard subq infusion was attempted. Her PFTs were normal before
> the infusion began.  Her FVC was actually 77%, but her FEV1 was 85% and
> FEF25-75 was 116%.  Her chest was clear and she did not get a bronchodilator
> treatment before the infusion began.  She received no pre-meds before the
> infusion was started. The plan was to give her 5 grams of Gammagard.  After
> 2 grams, she developed itching of the mouth and end expiratory wheeze.  The
> infusion was stopped and she felt a bit better after 60 minutes so the
> infusion was restarted. Wheezing worsened as did the itching.  At that time
> a serum tryptase was drawn which came back as 2.  When she was clearly
> wheezing, there was no change in her PFTs.
>
>
> Suggestions for a way to treat her?
>
>
> Thanks,
>
> Richard Wasserman
>
> Dallas
>
> --
> Richard L. Wasserman, MD, PhD
> DallasAllergyImmunology
> 7777 Forest Lane, Suite B-332
> Dallas, Texas 75230
> Office (972) 566-7788
> Fax (972) 566-8837
> Cell (214) 697-7211
>
> ---
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