Immunoglobulin Replacement Therapy for Primary Immunodeficiency

Immunology and Allergy Clinics of North America

Volumn 35, Issue 4, 2015, Pages 713-730

  Sriaroon, Panida ^a   Ballow, Mark ^a  

^a UNIVERSITY OF SOUTH FLORIDA   (United States)

Author keywords

Immunoglobulin replacement therapy; Intravenous immunoglobulin; IVIG; Primary immunodeficiency; SCIG; Subcutaneous immunoglobulin

Indexed keywords

IMMUNOGLOBULIN A ANTIBODY; IMMUNOGLOBULIN G; IMMUNOGLOBULIN;

ACUTE KIDNEY FAILURE; ACUTE LUNG INJURY; ALOPECIA; ANAPHYLAXIS; ANOREXIA; ARTHRALGIA; ASEPTIC MENINGITIS; BACTERIAL INFECTION; BLOOD CLOTTING DISORDER; BLOODSTREAM INFECTION; BRONCHOSPASM; CHILL; COUGHING; DIARRHEA; DIZZINESS; DRUG CONTRAINDICATION; DRUG INDICATION; DRUG INDUSTRY; DRUG SAFETY; DRUG TOLERABILITY; DYSESTHESIA; DYSPNEA; ERYTHEMA MULTIFORME; FATIGUE; FEVER; FLU LIKE SYNDROME; HEADACHE; HEART ARRHYTHMIA; HEART INFARCTION; HEART PALPITATION; HEMOLYSIS; HOT FLUSH; HUMAN; HYPERTENSION; HYPERVISCOSITY SYNDROME; HYPONATREMIA; HYPOTENSION; HYPOTHERMIA; IMMUNE DEFICIENCY; JOINT SWELLING; KIDNEY TUBULE DISORDER; MACULOPAPULAR RASH; MALAISE; MIGRAINE; MUSCLE CRAMP; MUSCULOSKELETAL PAIN; MYALGIA; NAUSEA; NERVE DEGENERATION; NEUTROPENIA; PATIENT MONITORING; PLEURA EFFUSION; PRIORITY JOURNAL; PRURITUS; REVIEW; RISK ASSESSMENT; SERUM SICKNESS; SHOCK; SKIN TINGLING; STEADY STATE; SUBSTITUTION THERAPY; TACHYCARDIA; THORAX PAIN; THROMBOEMBOLISM; TREATMENT DURATION; URTICARIA; UVEITIS; VASCULITIS; VOMITING; WEAKNESS; ADVERSE EFFECTS; COMPLICATION; IMMUNOLOGIC DEFICIENCY SYNDROMES; PASSIVE IMMUNIZATION; PHYSIOLOGIC MONITORING; PROCEDURES; SUBCUTANEOUS DRUG ADMINISTRATION; TREATMENT OUTCOME;

HUMANS; IMMUNIZATION, PASSIVE; IMMUNOGLOBULIN G; IMMUNOGLOBULINS; IMMUNOGLOBULINS, INTRAVENOUS; IMMUNOLOGIC DEFICIENCY SYNDROMES; INJECTIONS, SUBCUTANEOUS; MONITORING, PHYSIOLOGIC; TREATMENT OUTCOME;

EID: 84943816079     PISSN: 08898561     EISSN: 15578607     Source Type: Journal    
DOI: 10.1016/j.iac.2015.07.006     Document Type: Review

References (83)

1. Bruton O.C. Agammaglobulinemia. Pediatrics 1952, 9(6):722-728.
2. Lieberman P., Berger M. Intramuscular versus intravenous immunoglobulin replacement therapy and measurement of immunoglobulin levels during immunoglobulin replacement therapy. J Allergy Clin Immunol 2013, 1(6):705-706.
3. Eibl M.M. History of immunoglobulin replacement. Immunol Allergy Clin North Am 2008, 28(4):737-764. viii.
4. Berger M. Subcutaneous administration of IgG. Immunol Allergy Clin North Am 2008, 28(4):779-802. viii.
5. Orange J.S., Hossny E.M., Weiler C.R., et al. Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology. J Allergy Clin Immunol 2006, 117(4 Suppl):S525-S553.
6. Lucas M., Lee M., Lortan J., et al. Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. J Allergy Clin Immunol 2010, 125(6):1354-1360.e4.
7. Albin S., Cunningham-Rundles C. An update on the use of immunoglobulin for the treatment of immunodeficiency disorders. Immunotherapy 2014, 6(10):1113-1126.
8. Compagno N., Malipiero G., Cinetto F., et al. Immunoglobulin replacement therapy in secondary hypogammaglobulinemia. Front Immunol 2014, 5:626.
9. Ochs H.D., Fischer S.H., Wedgwood R.J., et al. Comparison of high-dose and low-dose intravenous immunoglobulin therapy in patients with primary immunodeficiency diseases. Am J Med 1984, 76(3A):78-82.
10. Stiehm E. Immunodeficiency disorders general conditions 1996, Saunders, Philadelphia (PA). 4th edition.
11. Roifman C.M., Levison H., Gelfand E.W. High-dose versus low-dose intravenous immunoglobulin in hypogammaglobulinaemia and chronic lung disease. Lancet 1987, 1(8541):1075-1077.
12. Eijkhout H.W., van Der Meer J.W., Kallenberg C.G., et al. The effect of two different dosages of intravenous immunoglobulin on the incidence of recurrent infections in patients with primary hypogammaglobulinemia. A randomized, double-blind, multicenter crossover trial. Ann Intern Med 2001, 135(3):165-174.
13. Orange J.S., Grossman W.J., Navickis R.J., et al. Impact of trough IgG on pneumonia incidence in primary immunodeficiency: a meta-analysis of clinical studies. Clin Immunol 2010, 137(1):21-30.
14. Bonagura V.R. Illustrative cases on individualizing immunoglobulin therapy in primary immunodeficiency disease. Ann Allergy Asthma Immunol 2013, 111(6 Suppl):S10-S13.
15. Khan S., Grimbacher B., Boecking C., et al. Serum trough IgG level and annual intravenous immunoglobulin dose are not related to body size in patients on regular replacement therapy. Drug Metab Lett 2011, 5(2):132-136.
16. Rocchio M.A., Hussey A.P., Southard R.A., et al. Impact of ideal body weight dosing for all inpatient i.v. immune globulin indications. Am J Health Syst Pharm 2013, 70(9):751-752.
17. Siegel J. Immunoglobulins and obesity: dosing. Considerations Pharmay Pract News 2013, 2013:40. Available at:, Accessed April 9, 2015. http://pharmacypracticenews.com/ViewArticle.aspx?d=Clinical&d_id=50&i=August+2013&i_id=985&a_id=23771.
18. Wasserman R.L. Progress in gammaglobulin therapy for immunodeficiency: from subcutaneous to intravenous infusions and back again. J Clin Immunol 2012, 32(6):1153-1164.
19. Misbah S.A. Effective dosing strategies for therapeutic immunoglobulin: managing wear-off effects in antibody replacement to immunomodulation. Clin Exp Immunol 2014, 178(Suppl 1):70-71.
20. Gardulf A., Hammarstrom L., Smith C.I. Home treatment of hypogammaglobulinaemia with subcutaneous gammaglobulin by rapid infusion. Lancet 1991, 338(8760):162-166.
21. Abrahamsen T.G., Sandersen H., Bustnes A. Home therapy with subcutaneous immunoglobulin infusions in children with congenital immunodeficiencies. Pediatrics 1996, 98(6 Pt 1):1127-1131.
22. Thomas M.J., Brennan V.M., Chapel H.H. Rapid subcutaneous immunoglobulin infusions in children. Lancet 1993, 342(8884):1432-1433.
23. Gaspar J., Gerritsen B., Jones A. Immunoglobulin replacement treatment by rapid subcutaneous infusion. Arch Dis Child 1998, 79(1):48-51.
24. Abolhassani H., Sadaghiani M.S., Aghamohammadi A., et al. Home-based subcutaneous immunoglobulin versus hospital-based intravenous immunoglobulin in treatment of primary antibody deficiencies: systematic review and meta analysis. J Clin Immunol 2012, 32(6):1180-1192.
25. Berger M., Jolles S., Orange J.S., et al. Bioavailability of IgG administered by the subcutaneous route. J Clin Immunol 2013, 33(5):984-990.
26. Berger M., Rojavin M., Kiessling P., et al. Pharmacokinetics of subcutaneous immunoglobulin and their use in dosing of replacement therapy in patients with primary immunodeficiencies. Clin Immunol 2011, 139(2):133-141.
27. Jolles S., Bernatowska E., de Gracia J., et al. Efficacy and safety of Hizentra((R)) in patients with primary immunodeficiency after a dose-equivalent switch from intravenous or subcutaneous replacement therapy. Clin Immunol 2011, 141(1):90-102.
28. Gardulf A., Nicolay U., Asensio O., et al. Rapid subcutaneous IgG replacement therapy is effective and safe in children and adults with primary immunodeficiencies-a prospective, multi-national study. J Clin Immunol 2006, 26(2):177-185.
29. Shapiro R.S. Subcutaneous immunoglobulin therapy given by subcutaneous rapid push vs infusion pump: a retrospective analysis. Ann Allergy Asthma Immunol 2013, 111(1):51-55.
30. Shapiro R.S. Subcutaneous immunoglobulin: rapid push vs infusion pump in pediatrics. Pediatr Allergy Immunol 2013, 24(1):49-53.
31. Wasserman R.L., Melamed I., Stein M.R., et al. Recombinant human hyaluronidase-facilitated subcutaneous infusion of human immunoglobulins for primary immunodeficiency. J Allergy Clin Immunol 2012, 130(4):951-957.e11.
32. Bonilla F.A., Bernstein I.L., Khan D.A., et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol 2005, 94(5 Suppl 1):S1-S63.
33. Orange J.S., Ballow M., Stiehm E.R., et al. Use and interpretation of diagnostic vaccination in primary immunodeficiency: a working group report of the Basic and Clinical Immunology Interest Section of the American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol 2012, 130(3 Suppl):S1-S24.
34. Berger M. Adverse effects of IgG therapy. J Allergy Clin Immunol 2013, 1(6):558-566.
35. Peter J.G., Chapel H. Immunoglobulin replacement therapy for primary immunodeficiencies. Immunotherapy 2014, 6(7):853-869.
36. Ballow M. Safety of IGIV therapy and infusion-related adverse events. Immunol Res 2007, 38(1-3):122-132.
37. Lederman H.M., Roifman C.M., Lavi S., et al. Corticosteroids for prevention of adverse reactions to intravenous immune serum globulin infusions in hypogammaglobulinemic patients. Am J Med 1986, 81(3):443-446.
38. Ballow M. Clinical and investigational considerations for the use of IGIV therapy. Am J Health Syst Pharm 2005, 62(16 Suppl 3):S12-S18. [quiz: S19-21].
39. Berger M., Pinciaro P.J., Flebogamma I. Safety, efficacy, and pharmacokinetics of Flebogamma 5% [immune globulin intravenous (human)] for replacement therapy in primary immunodeficiency diseases. J Clin Immunol 2004, 24(4):389-396.
40. Finkel A.G., Howard J.F., Mann J.D. Successful treatment of headache related to intravenous immunoglobulin with antimigraine medications. Headache 1998, 38(4):317-321.
41. Sekul E.A., Cupler E.J., Dalakas M.C. Aseptic meningitis associated with high-dose intravenous immunoglobulin therapy: frequency and risk factors. Ann Intern Med 1994, 121(4):259-262.
42. Roberton D.M., Hosking C.S. Use of methylprednisolone as prophylaxis for immediate adverse infusion reactions in hypogammaglobulinaemic patients receiving intravenous immunoglobulin: a controlled trial. Aust Paediatr J 1988, 24(3):174-177.
43. Brennan V.M., Salome-Bentley N.J., Chapel H.M., et al. Prospective audit of adverse reactions occurring in 459 primary antibody-deficient patients receiving intravenous immunoglobulin. Clin Exp Immunol 2003, 133(2):247-251.
44. Rachid R., Bonilla F.A. The role of anti-IgA antibodies in causing adverse reactions to gamma globulin infusion in immunodeficient patients: a comprehensive review of the literature. J Allergy Clin Immunol 2012, 129(3):628-634.
45. Burks A.W., Sampson H.A., Buckley R.H. Anaphylactic reactions after gamma globulin administration in patients with hypogammaglobulinemia. Detection of IgE antibodies to IgA. N Engl J Med 1986, 314(9):560-564.
46. Bjorkander J., Hammarstrom L., Smith C.I., et al. Immunoglobulin prophylaxis in patients with antibody deficiency syndromes and anti-IgA antibodies. J Clin Immunol 1987, 7(1):8-15.
47. Stiehm E.R. Adverse effects of human immunoglobulin therapy. Transfus Med Rev 2013, 27(3):171-178.
48. Eijkhout H.W., van den Broek P.J., van der Meer J.W. Substitution therapy in immunodeficient patients with anti-IgA antibodies or severe adverse reactions to previous immunoglobulin therapy. Neth J Med 2003, 61(6):213-217.
49. Sundin U., Nava S., Hammarstrom L. Induction of unresponsiveness against IgA in IgA-deficient patients on subcutaneous immunoglobulin infusion therapy. Clin Exp Immunol 1998, 112(2):341-346.
50. Brannagan T.H., Nagle K.J., Lange D.J., et al. Complications of intravenous immune globulin treatment in neurologic disease. Neurology 1996, 47(3):674-677.
51. Scribner C.L., Kapit R.M., Phillips E.T., et al. Aseptic meningitis and intravenous immunoglobulin therapy. Ann Intern Med 1994, 121(4):305-306.
52. Kato E., Shindo S., Eto Y., et al. Administration of immune globulin associated with aseptic meningitis. JAMA 1988, 259(22):3269-3271.
53. Mathy I., Gille M., Van Raemdonck F., et al. Neurological complications of intravenous immunoglobulin (IVIg) therapy: an illustrative case of acute encephalopathy following IVIg therapy and a review of the literature. Acta Neurol Belg 1998, 98(4):347-351.
54. Cantu T.G., Hoehn-Saric E.W., Burgess K.M., et al. Acute renal failure associated with immunoglobulin therapy. Am J kidney Dis 1995, 25(2):228-234.
55. Zhang R., Szerlip H.M. Reemergence of sucrose nephropathy: acute renal failure caused by high-dose intravenous immune globulin therapy. South Med J 2000, 93(9):901-904.
56. Centers for Disease Control and Prevention (CDC) Renal insufficiency and failure associated with immune globulin intravenous therapy-United States, 1985-1998. MMWR Morb Mortal Wkly Rep 1999, 48(24):518-521.
57. Bonilla F.A. Adverse effects of immunoglobulin G therapy: thromboembolism and haemolysis. Clin Exp Immunol 2014, 178(Suppl 1):72-74.
58. Daw Z., Padmore R., Neurath D., et al. Hemolytic transfusion reactions after administration of intravenous immune (gamma) globulin: a case series analysis. Transfusion 2008, 48(8):1598-1601.
59. Kahwaji J., Barker E., Pepkowitz S., et al. Acute hemolysis after high-dose intravenous immunoglobulin therapy in highly HLA sensitized patients. Clin J Am Soc Nephrol 2009, 4(12):1993-1997.
60. Morgan S., Sorensen P., Vercellotti G., et al. Haemolysis after treatment with intravenous immunoglobulin due to anti-A. Transfus Med 2011, 21(4):267-270.
61. Bellac C.L., Polatti D., Hottiger T., et al. Anti-A and anti-B haemagglutinin levels in intravenous immunoglobulins: are they on the rise? A comparison of four different analysis methods and six products. Biologicals 2014, 42(1):57-64.
62. Welles C.C., Tambra S., Lafayette R.A. Hemoglobinuria and acute kidney injury requiring hemodialysis following intravenous immunoglobulin infusion. Am J kidney Dis 2010, 55(1):148-151.
63. Strategies to address hemolytic complications of immune globulin infusions. Workshop on Risk Mitigation Measure Strategies. 2014. Available at: . Accessed April 9, 2015. http://www.fda.gov/downloads/BiologicsBloodVaccines/NewsEvents/WorkshopsMeetingsConferences/UCM387078.pdf.
64. Robertson V.M., Dickson L.G., Romond E.H., et al. Positive antiglobulin tests due to intravenous immunoglobulin in patients who received bone marrow transplant. Transfusion 1987, 27(1):28-31.
65. Wilson J.R., Bhoopalam H., Fisher M. Hemolytic anemia associated with intravenous immunoglobulin. Muscle Nerve 1997, 20(9):1142-1145.
66. Pintova S., Bhardwaj A.S., Aledort L.M. IVIG-a hemolytic culprit. N Engl J Med 2012, 367(10):974-976.
67. Caress J.B., Hobson-Webb L., Passmore L.V., et al. Case-control study of thromboembolic events associated with IV immunoglobulin. J Neurol 2009, 256(3):339-342.
68. Caress J.B., Cartwright M.S., Donofrio P.D., et al. The clinical features of 16 cases of stroke associated with administration of IVIg. Neurology 2003, 60(11):1822-1824.
69. Go R.S., Call T.G. Deep venous thrombosis of the arm after intravenous immunoglobulin infusion: case report and literature review of intravenous immunoglobulin-related thrombotic complications. Mayo Clin Proc 2000, 75(1):83-85.
70. Rajabally Y.A., Kearney D.A. Thromboembolic complications of intravenous immunoglobulin therapy in patients with neuropathy: a two-year study. J Neurol Sci 2011, 308(1-2):124-127.
71. Reinhart W.H., Berchtold P.E. Effect of high-dose intravenous immunoglobulin therapy on blood rheology. Lancet 1992, 339(8794):662-664.
72. Dalakas M.C. High-dose intravenous immunoglobulin and serum viscosity: risk of precipitating thromboembolic events. Neurology 1994, 44(2):223-226.
73. Crouch E.D., Watson L.E. Intravenous immunoglobulin-related acute coronary syndrome and coronary angiography in idiopathic thrombocytopenic purpura-a case report and literature review. Angiology 2002, 53(1):113-117.
74. FDA Safety Communication: Updated information on the risks of thrombosis and hemolysis potentially related to administration of intravenous, subcutaneous and intramuscular human immune globulin products. 2012. Available at: . Accessed April 9, 2015. http://www.fda.gov/biologicsbloodvaccines/safetyavailability/ucm327934.htm.
75. Ovanesov MV. Laboratory investigation that identified the cause of thromboembolic events in patients receiving immunoglobulin treatments. 2012. Available at: . Accessed April 9, 2015. http://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/ScienceBoardtotheFoodandDrugAdministration/UCM321916.pdf.
76. Roemisch J, Kaar W, Zoechling A, et al. Identification of activated FXI as the major biochemical root cause in IVIG batches associated with thromboembolic events. Analytical and experimental approaches resulting in corrective and preventive measures implemented into the Octagam manufacturing process. WebmedCentral IMMUNOTHERAPY. 2011;2(6). Available at: . Accessed April 9, 2015. http://www.webmedcentral.com/article_view/2002.
77. Ballow M. Intravenous immunoglobulins: clinical experience and viral safety. J Am Pharm Assoc 2002, 42(3):449-458. [quiz: 458-9].
78. Ochs H.D., Gupta S., Kiessling P., Subcutaneous IgG Study Group, et al. Safety and efficacy of self-administered subcutaneous immunoglobulin in patients with primary immunodeficiency diseases. J Clin Immunol 2006, 26(3):265-273.
79. Hagan J.B., Fasano M.B., Spector S., et al. Efficacy and safety of a new 20% immunoglobulin preparation for subcutaneous administration, IgPro20, in patients with primary immunodeficiency. J Clin Immunol 2010, 30(5):734-745.
80. Wasserman R.L., Irani A.M., Tracy J., et al. Pharmacokinetics and safety of subcutaneous immune globulin (human), 10% caprylate/chromatography purified in patients with primary immunodeficiency disease. Clin Exp Immunol 2010, 161(3):518-526.
81. Wasserman R.L., Melamed I., Kobrynski L., et al. Efficacy, safety, and pharmacokinetics of a 10% liquid immune globulin preparation (GAMMAGARD LIQUID, 10%) administered subcutaneously in subjects with primary immunodeficiency disease. J Clin Immunol 2011, 31(3):323-331.
82. Berger M. Subcutaneous immunoglobulin replacement in primary immunodeficiencies. Clin Immunol 2004, 112(1):1-7.
83. Wasserman R.L. Common infusion-related reactions to subcutaneous immunoglobulin therapy: managing patient expectations. Patient Prefer Adherence 2008, 2:163-166.