rapid infusion of immune globulin intravenous (human) in primary immunodeficiency patients

Conditions: Immunologic Deficiency Syndrome; Agammaglobulinemia; Severe Combined Immunodeficiency; Wiskott-Aldrich Syndrome; Common Variable Immunodeficiency

Summary: the objective of this study is to determine if the safety and tolerability of immune globulin intravenous (human), 10% caprylate/chromatography (igiv-c)purified is similar when infused at two different infusion rates. the primary objective is to compare the incidence and severity of all infusion related adverse events when igiv-c, 10% is administered at a rate of 0.14 ml/kg/min compared to a rate of 0.08 ml/kg/min after a single daily infusion.

this is a prospective, single blind, randomized, multi-center cross-over trial in patients with primary immune deficiency. patients with a confirmed diagnosis of primary immune deficiency will be treated with two daily infusions given 3-4 weeks apart at the fixed individual igiv dose regimen (400-600 mg/kg) established prior to entry into the study. any subject with an established dose in the range of 200-399 mg/kg will be assigned to receive 400 mg/kg during the course of the study during the same dosing schedule established prior to entry into the study. after a screening period lasting not more than four weeks, patients will be randomized into one of two cross-over groups. patients randomized to group 1 will receive their first igiv-c, 10% dose at a rate of 0.08 ml/kg/min and their second infusion at a rate of 0.14 ml/kg/min, whereas patients randomized to group 2 will receive igiv-c, 10% at a rate of 0.14 ml/kg/min on the first infusion day and then 0.08 ml/kg/min on the second infusion day. all patients just prior to each igiv-c, 10% infusion will receive the same volume of 5% dextrose as calculated for their igiv-c, 10% infusion and given at a target rate according to the schema below. group 1: * infusion #1 (week 0)dextrose (0.14 ml/kg/min), then igiv-c, 10% (0.08 ml/kg/min) * infusion #2 (week 3-4)dextrose (0.08 ml/kg/min), then igiv-c, 10% (0.14 ml/kg/min) group 2: * infusion #1 (week 0)dextrose (0.08 ml/kg/min), then igiv-c, 10% (0.14 ml/kg/min) * infusion #2 dextrose (0.14 ml/kg/min), then igiv-c, 10% (0.08 ml/kg/min)

Eligibility criteria:
inclusion criteria: * confirmed diagnosis of primary immune deficiency and medical records available for retrospective review for at least 3 months prior to entry into the trial * signed an informed consent written informed consent prior to initiation of any study related procedures * receiving regular infusions of igiv at a fixed interval and dosage (in the range of 200-600 mg/kg given every 3-4 weeks) for at least three months prior to entry into the trial. patients who are currently receiving less than 400 mg/kg are eligible for this trial and will be at the time of study enrollment be treated at 400 mg/kg exclusion criteria: * history or suspicion of significant allergic reaction to intravenous immune globulin, and/or blood products * documented history of selective iga deficiency (serum level \<5.0 mg/dl) and known antibodies to iga * isolated igg subclass deficiency with a normal total serum igg level * other conditions which may interfere with the trial, include the patients demeanor or mental ability to follow instruction. * pretreatment with anti-pyretics or anti-histamines * congestive heart failure (new york heart association stage greater than class ii) * renal insufficiency (creatinine \>2.5 mg/dl) * conditions whose symptoms and effects could alter protein catabolism and/or igg utilization (e.g. protein-losing enteropathies, nephrotic syndrome) * pretreatment routinely required to control/ameliorate igiv infusion-related adverse events (aes) * any patient who requires igiv dosing more frequently than every 3 weeks to maintain adequate trough levels * women of child bearing potential who do not practice adequate contraception (i.e. chemical or mechanical methods) and pregnant or lactating females

Keywords: primary immune deficiency igiv immunoglobulin g