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Guidelines on the Use of Intravenous Immune Globulin for Hematologic Conditions

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Guidelines on the Use of Intravenous Immune Globulin for Hematologic Conditions David Anderson, Kaiser Ali, Victor Blanchette, Melissa Brouwers, Stephen Couban, Paula Radmoor, Lothar Huebsch, Heather Hume, Anne McLeod, Ralph Meyer, Catherine Moltzan, Susan Nahirniak, Stephen Nantel, Graham Pineo, and Gail Rock Canada’s per capita use of intravenous immune basis for making recommendations to provincial and globulin (IVIG) grew by approximately 115% between territorial health ministries regarding IVIG use man- 1998 and 2006, making Canada one of the world’s agement. Specific recommendations for routine use highest per capita users of IVIG. It is believed that of IVIG were made for 7 conditions including acquired most of this growth is attributable to off-label usage. red cell aplasia; acquired hypogammaglobulinemia To help ensure IVIG use is in keeping with an (secondary to malignancy); fetal-neonatal alloim- evidence-based approach to the practice of medicine, mune thrombocytopenia; hemolytic disease of the the National Advisory Committee on Blood and Blood newborn; HIV-associated thrombocytopenia; idio- Products of Canada (NAC) and Canadian Blood pathic thrombocytopenic purpura; and posttransfu- Services convened a panel of national experts to sion purpura. Intravenous immune globulin was not develop an evidence-based practice guideline on the recommended for use, except under certain life- use of IVIG for hematologic conditions. The mandate threatening circumstances, for 8 conditions including of the expert panel was to review evidence regarding acquired hemophilia; acquired von Willebrand dis- use of IVIG for 18 hematologic conditions and ease; autoimmune hemolytic anemia; autoimmune formulate recommendations on IVIG use for each. A neutropenia; hemolytic transfusion reaction; hemo- panel of 13 clinical experts and 1 expert in practice lytic transfusion reaction associated with sickle cell guideline development met to review the evidence disease; hemolytic uremic syndrome/thrombotic and reach consensus on the recommendations for the thrombocytopenic purpura; and viral-associated use of IVIG. The primary sources used by the panel hemophagocytic syndrome. Intravenous immune were 3 recent evidence-based reviews. Recommen- globulin was not recommended for 2 conditions dations were based on interpretation of the available (aplastic anemia and hematopoietic stem cell trans- evidence and where evidence was lacking, consensus plantation) and was contraindicated for 1 condition of expert clinical opinion. A draft of the practice (heparin-induced thrombocytopenia). For most he- guideline was circulated to hematologists in Canada matologic conditions reviewed by the expert panel, for feedback. The results of this process were routine use of IVIG was not recommended. Develop- reviewed by the expert panel, and modifications to ment and dissemination of evidence-based guidelines the draft guideline were made where appropriate. may help to facilitate appropriate use of IVIG. This practice guideline will provide the NAC with a A 2007 Published by Elsevier Inc. DESCRIPTION OF INTRAVENOUS indications, including a variety of immunological IMMUNE GLOBULIN disorders, hematological conditions, and neurolog- NTRAVENOUS IMMUNE GLOBULIN ical diseases. Health Canada has not evaluated the I (IVIG) is a fractionated blood product consist- efficacy and risk of using a licensed IVIG product ing of concentrated immunoglobulin, primarily in the treatment of off-label clinical indications. immunoglobulin G (IgG), derived from human Nevertheless, some of these applications have a plasma in pools of 3000 to 10,000 plus donors. reasonably strong foundation in the medical Intravenous immune globulin was first introduced literature, whereas others have a less conclusive in the early 1980s for the treatment of primary or even no basis in evidence. humoral immunodeficiencies and is currently licensed by Health Canada for treatment of primary From the IVIG Hematology and Neurology Expert Panels. and secondary immunodeficiency diseases, allo- Address reprint requests to David Anderson, MD, FRCPC, genic bone marrow transplantation, chronic B-cell Professor of Medicine and Pathology, QEII Health Sciences lymphocytic leukemia, pediatric human immuno- Centre and Dalhousie University, Room 430, Bethune Bldg., deficiency virus (HIV)-infection, and idiopathic 1172 Tower Road Halifax, Nova Scotia, Canada V3H 1V8. E-mail: [email protected] thrombocytopenic purpura. 0887-7963/07/$ – see front matter In addition to its licensed indications, IVIG is n 2007 Published by Elsevier Inc. used to treat a growing range of boff-labelQ doi:10.1016/j.tmrv.2007.01.001 Transfusion Medicine Reviews, Vol 21, No 2, Suppl 1 (April), 2007: pp S9-S56 S9 S10 ANDERSON ET AL In appropriately selected patients and clinical production and product withdrawals caused by the settings, IVIG therapy can be lifesaving. However, need for US-based plasma fractionators to comply there are risks and significant costs associated with with more stringent US Food and Drug Adminis- IVIG. This provides a strong incentive to ensure tration requirements. Although such a severe that IVIG is prescribed only for appropriate clinical shortage has not recurred, the cost of IVIG has indications where there is a known benefit.1-3 continued to rise. This has led to the adoption of various approaches to control IVIG use in several RISKS ASSOCIATED WITH IVIG countries, in particular in Canada and Australia. The rate of systemic reactions to IVIG infusion Intravenous immune globulin is an expensive is usually reported to be in the 3% to 15% range. therapeutic alternative in disease states where other These reactions are typically self-limited, of mild interventions may be possible or where its efficacy to moderate severity, and can often be avoided by is questionable. Intravenous immune globulin rep- reducing the rate of infusion during subsequent resents the single largest component (approximately transfusions of IVIG. However, there is a paucity one third) of Canadian Blood Services (CBS) of published reports of prospectively collected data plasma protein products budget, which, in turn, on the adverse event rate associated with IVIG. represents approximately half of the CBS total Moreover, each brand of IVIG may have unique budget. Because Canada is not self-sufficient in tolerability and safety profiles because of propriety plasma, IVIG used in this country is manufactured differences in the manufacturing methods. from plasma donated either voluntarily in Canada or A recent review by Pierce and Jain4 found that a by paid donors in the United States. The CBS significant number of IVIG-associated serious ensures a supply of IVIG for Canada through multi- adverse events affecting renal, cardiovascular, year agreements with manufacturers, which provide central nervous system (CNS), integumentary, stability in pricing and purchase volumes. Funding and hematologic systems have been reported. In for IVIG comes from provincial and territorial view of the seriousness of potential adverse events health budgets as part of their payment to CBS; and current lack of data surrounding their frequen- thus, this charge is not directly visible to either cy, the review concluded that clinicians should patient or provider. Provinces and territories are limit their prescription of IVIG to conditions for charged for the actual amount of product used in which efficacy is supported by adequate and well- their province/territory. There are also direct hospi- controlled clinical trials. tal costs, as IVIG must be administered intrave- The risk of infectious complications from IVIG is nously over several hours. extremely low. The requirements for donor screen- Intravenous immune globulin currently costs ing and transmissible disease testing of input plasma between $51 and $64 per gram (all estimates in are stringent. In addition, the IVIG manufacturing Canadian dollars), but in past years, with a less process itself includes at least 1 and usually 2 steps favorable US exchange rate, the cost has been of viral inactivation or removal to protect against as high as $75 to $80. The cost of one infusion of infectious agents that might be present despite 1 g/kg of IVIG for a 70-kg adult is approximately screening procedures. Hepatitis B virus and HIV $4000 (see Table 1). have never been transmitted through IVIG. There Canada’s per capita use of IVIG grew by has been no reported transmission of hepatitis C approximately 83% between 1998 and 2004 (and virus from any product used in Canada, and there is another 18% between 2004 and 2006), making no known case of Creutzfeldt-Jakob disease or Canada one of the highest per capita users of IVIG variant Creutzfeldt-Jakob disease transmission due in the world. It is believed that most of the growth to IVIG transfusion. Nevertheless, IVIG is a product in use is attributable to off-label usage. made from large pools of human plasma, and it is not possible to claim with certainty that there is no IMPETUS AND MANDATE TO DEVELOP AN IVIG risk of infectious disease transmission. PRACTICE GUIDELINE In view of the escalating costs, potential for COSTS OF IVIG shortages, and growing off-label usage associated In 1997 there was a worldwide IVIG shortage. with IVIG, over the past 5 years there have been The shortage was caused primarily by disruption of several initiatives in Canada aimed at ensuring IVIG USE OF INTRAVENOUS IMMUNE GLOBULIN FOR HEMATOLOGIC CONDITIONS S11 Table 1. Examples of the Cost of IVIG (NAC), an advisory group to provincial and Cost of IVIG4 territorial Deputy Ministers of Health and Canadi- Patient Schedule 0.5 g/kg 1.0 g/kg 2.0 g/kg an Blood Services regarding blood use manage- 20-kg child 1 dose $550 $1100 $2200 ment issues, has been working on the development 1 Â monthly $6600 $13,200 $26,400 of an interprovincial collaborative framework for for 1 y IVIG use management. To facilitate this objective, 1 Â 3wk $9350 $18,700 $37,400 for 1 y the NAC and CBS convened a panel of national 70-kg adult 1 dose $2000 $4000 $8000 experts to develop an evidence-based practice 1 Â monthly $24,000 $48,000 $96,000 guideline on the use of IVIG for 18 hematologic for 1 y conditions.
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