Infusion parameters, safety, and practical guidance for the manual administration of subcutaneous immunoglobulin 20% (Ig20Gly).

Primary immunodeficiency diseases (PIDs), also referred to as inborn errors of immunity, constitute a group of genetic conditions that affect the immune system. The current standard of care for patients with PIDs is lifelong immunoglobulin replacement therapy, delivered by intravenous (IVIG) or subcutaneous (SCIG) infusion. Immune globulin subcutaneous (human) 20% solution stabilized with glycine (Ig20Gly) is indicated as a replacement therapy for PIDs in adults and children of any age in Europe and in patients aged 2 years and above in the USA. Typically, Ig20Gly is administered using an infusion pump; however, delivery of Ig20Gly by manual administration has recently been approved in Europe. Practical recommendations on the use of Ig20Gly manual administration are lacking; this review therefore aims to provide guidance for use of this method of administration. Additionally, we summarize the infusion parameters, safety, patient-reported outcomes, and economic benefits associated with Ig20Gly manual administration. Manual administration of Ig20Gly was shown to permit faster rates of infusion than administration via infusion pump. Patients typically infused at two or fewer infusion sites with manual administration of Ig20Gly. Safety and tolerability profiles were similar for Ig20Gly manual administration and administration by infusion pump. Overall, there were comparable levels of patient satisfaction with manual administration and infusion pump, with patient preference deemed to be a key determinator of success for either method of administration. Economic studies identified cost savings for the healthcare system through manual administration compared with IVIG or SCIG infusion by infusion pump because of the reduced equipment costs and nurse support. For infusion of Ig20Gly by manual administration, a syringe and butterfly needle are used; patients are advised to start infusion at 1-2 mL/min to prevent discomfort. Overall, manual administration of Ig20Gly offers an effective and well-tolerated alternative to administration by infusion pump.

A National Survey of Hereditary Angioedema and Acquired C1 Inhibitor Deficiency in the United Kingdom.

BACKGROUND: Detailed demographic data on people with hereditary angioedema (HAE) and acquired C1 inhibitor deficiency in the United Kingdom are relatively limited. Better demographic data would be beneficial in planning service provision, identifying areas of improvement, and improving care. OBJECTIVE: To obtain more accurate data on the demographics of HAE and acquired C1 inhibitor deficiency in the United Kingdom, including treatment modalities and services available to patients. METHODS: A survey was distributed to all centers in the United Kingdom that look after patients with HAE and acquired C1 inhibitor deficiency to collect these data. RESULTS: The survey identified 1152 patients with HAE-1/2 (58% female and 92% type 1), 22 patients with HAE with normal C1 inhibitor, and 91 patients with acquired C1 inhibitor deficiency. Data were provided by 37 centers across the United Kingdom. This gives a minimum prevalence of 1:59,000 for HAE-1/2 and 1:734,000 for acquired C1 inhibitor deficiency in the United Kingdom. A total of 45% of patients with HAE were on long-term prophylaxis (LTP) with the most used medication being danazol (55% of all patients on LTP). Eighty-two percent of patients with HAE had a home supply of acute treatment with C1 inhibitor or icatibant. A total of 45% of patients had a supply of icatibant and 56% had a supply of C1 inhibitor at home. CONCLUSIONS: Data obtained from the survey provide useful information about the demographics and treatment modalities used in HAE and acquired C1 inhibitor deficiency in the United Kingdom. These data are useful for planning service provision and improving services for these patients.

Successful desensitization to immunoglobulin A in a case of transfusion-related anaphylaxis.

BACKGROUND: A history of anaphylaxis after transfusion of immunoglobulin A (IgA)-containing blood products in selective IgA-deficient (sIgAD) patients can be a major problem, particularly in emergencies, when large quantities of blood products are required. CASE REPORT: A 19-year-old woman with end-stage Type 2 autoimmune hepatitis required liver transplantation as her only remaining treatment option. However, she also had sIgAD, anti-IgA antibodies, and episodes of anaphylaxis after receiving IgA-containing blood products. Liver transplantation would have been extremely challenging due to the difficulty of obtaining sufficient blood products from suitable IgA-deficient donors. Hence, it became imperative to devise a protocol to desensitize her to IgA-containing blood products. RESULTS: Using a continuous infusion of an IgA-enriched (6 mg/mL IgA) immunoglobulin preparation with gradual increases in concentration, she was successfully desensitized to IgA. Consequently, she was able to receive standard platelets, fresh-frozen plasma, and red blood cells with no complications. CONCLUSION: This approach could prove very useful in similar cases that may require administration of large quantities of blood products particularly in emergency lifesaving circumstances.