Reactions 1463, p21-22 - 3 Aug 2013

SImmune globulin

Anaphylactoid reaction, with subsequentdesensitisation: case report

A 28-year-old woman with common variableimmunodeficiency (CVID) developed an anaphylactoidreaction while receiving immune globulin; she later underwentsuccessful desensitisation.

The woman, who had acquired hypogammaglobulinaemia,required antibody supplementation for recurrent infections.While receiving her first IV infusion of immune globulin,containing IgA 1.5 mg/mL [dosage not stated], she reportedabdominal pain and nausea [duration of treatment to reactiononset not stated]. She also had a generalised rash, tachycardia,decreased BP and a respiratory rate of 36 breaths/minute. Halfan hour later, she developed chills followed by a fever.

Immune globulin was withdrawn, and the woman receivedclemastine, prednisolone sodium succinate and sodiumchloride. Her condition improved within a few hours, and shewas discharged 24 hours later. Tests showed elevated levels ofneutrophil-derived serum elastase during the anaphylactoidreaction, while serum tryptase and complement activationproducts were not increased. Analysis of preinfusion serumsamples revealed the presence of anti-IgA antibodies of thesubclass IgG1.

Two weeks later, the woman received IM 16% immuneglobulin solution in gradually increasing dosages for 3 days.She continued to receive weekly IM immune globulin until2 months later, when she received IV immune globulinwithout complications. At that time, her anti-IgA antibodieshad disappeared. Genetic analysis identified a novel splicevariant of her neutrophil Fcγ receptor IIa that contained anexpressed cryptic exon 6* (FcγRIIaexon6*). Neutrophils thatexpressed this novel splice variant were found to haveincreased sensitivity to small immune complexes.

Author comment: "The index patient with CVID had highlevels of anti-IgA antibodies of the IgG1 subclass, and the IV[immune globulin] preparation used contains up to 1.5 mg/LIgA. Therefore small immune complexes might have formedin vivo that bound to FcγRIIaexon6* on neutrophils and hencecaused the severe side effects observed in this patient oninfusion with IV [immune globulin]."van der Heijden J, et al. A novel splice variant of FcRIIa: a risk factor foranaphylaxis in patients with hypogammaglobulinemia. Journal of Allergy andClinical Immunology 131: 1408-1416.e5, No. 5, May 2013. Available from: URL:http://dx.doi.org/10.1016/j.jaci.2013.02.009 - Netherlands 803090774

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Reactions 3 Aug 2013 No. 14630114-9954/13/1463-0001/$14.95 Adis © 2013 Springer International Publishing AG. All rights reserved