Allergic reactions, including anaphylaxis, are a well-known complication in some patients who receive blood/plasma transfusions and have an immunoglobulin A (IgA) deficiency. Even patients with panhypogammaglobulinemia and deficient vaccine response may experience anaphylactic episodes probably because the infused immunoglobulins may induce antibody-mediated reactions when these patients receive the treatment (IgIV).
We present a 26-year-old woman with a history of Burkitt’s lymphoma treated in 2014 with chemotherapy. A profound hypogammaglobulinemia was generated over the next few years with a progressive decrease in immunoglobulins from 2015 until the total absence of IgA and IgM (2018) and IgG (2020). During that period, she had to be assisted in the emergency room frequently for many respiratory infections, including pneumonia. In 2020, after symptomatic and progressive bronchiectasis with severe hemoptysis, immunoglobulins were recommended. The first infusion of intravenous immunoglobulins was administered. Close to the end of infusion, she suffered a facial edema and acute bronchospasm that was resolved after suspending the infusion and administering treatment. A new slow infusion with a classical pretreatment was attempted in November 2020 with a recurrence of symptoms a few minutes after the start; the patient was ruled out for new infusions. Later she was referred to the Primary Immunodeficiency Unit in December 2021.
Lymphocytes 2300 cells/dl (CD69+ < 10 and CD3+2200); IgG < 3 mg/dL; IgA < 6 mg/dL; and IgM < 3 mg/dl. Antibodies post-vaccine: anti-diphtheria toxoid < 0.01 IU/ml, anti-pneumococcus < 3.33 mg/L, anti-salmonella < 7.40 U/ml, anti-tetanus toxoid < 0.01 IU/ml, anti-COVID IgA antibodies 0.30 (OD), and anti-COVID IgG antibodies 0.10. The COVID vaccine response study was positive for DTH (delayed type hypersensitivity). Anti-IgA antibodies were absent.
A desensitization program was designed using a variant of Castells' 12-step desensitization protocol (Castells M., Brigham and Women's Hospital, Harvard Medical School, Boston, MA) at the Hematology Day Hospital with full pretreatment and culminated with 20 g of intravenous immunoglobulin. The protocol was developed and successfully completed with only a slight subsequent headache. Since then, the same regimen was scheduled every 4 weeks with an improvement in her symptoms and quality of life.
