Efficacy and Safety Study of Kedrion IVIG 10% to Treat Subjects With Primary Immunodeficiency (PID)

Inclusion Criteria:

• Confirmed clinical diagnosis of a Primary Immunodeficiency Disease
• Male or female, ages 2 to 70 years
• Received 300-900 mg/kg of a licensed IVIG therapy at 21 or 28 day intervals for atleast 3 months prior to this study
• 2 documented IgG trough levels of ≥ 5 g/L are obtained at two infusion cycles (21 or 28 days) within 12 months (one must be within 6 months) prior to study enrolment
• Non-pregnant females of child-bearing potential who agree to use adequate birthcontrol during the study
• Subject is willing to comply with the protocol
• Authorization to access personal health information.
• Signed the informed consent form and a child assent form, if appropriate.
• If currently participating in a clinical trial with another experimental IVIG may beenrolled if they have received stable IVIG therapy for at least 3 infusion cyclesprior to receiving Kedrion IVIG 10% and all inclusion and exclusion criteria aresatisfied
• If currently participating in a trial of SCIG can be enrolled if they are switched toIVIG for three infusion cycles (21 or 28 days) prior to enrolment in this study

Exclusion Criteria:

• Has secondary immunodeficiency.
• Newly diagnosed and has not been treated with immunoglobulin or has been diagnosedwith dysgammaglobulinemia or isolated IgG subclass deficiency.
• Has a history of repeated reactions or hypersensitivity to IVIG or other injectableforms of IgG.
• Has a history of thrombotic events defined by at least 1 event in subject's lifetime.
• Has IgA deficiency and is known to have antibodies to IgA.
• Has received blood products other than human albumin or human immunoglobulin within 12months prior to enrolment.
• Has significant protein losing enteropathy, nephrotic syndrome or lymphangiectasia.
• Has an acute infection as documented by culture or diagnostic imaging and/or a bodytemperature exceeding 38.5 °C (101.3 °F) within 7 days prior to screening
• Has a known history or is positive at enrolment for human immunodeficiency virus (HIV)type 1 by NAT, hepatitis B virus (HBsAg and NAT), hepatitis C virus (by NAT), orhepatitis A virus (by NAT).
• Has levels of alanine aminotransferase (ALT) or aspartate aminotransferase (AST) > 2.5times of the upper limit of normal for the laboratory designated for the study.
• Has an implanted venous access device
• Has profound anemia or persistent severe neutropenia (≤ 1000 neutrophils per mm3)orlymphopenia of less than 500 cells per microliter.
• Has a severe chronic condition such as renal failure (creatinine concentration > 2.0times the upper limit of normal) with proteinuria, congestive heart failure (New YorkHeart Association III/IV), cardiomyopathy, cardiac arrhythmia associated withthromboembolic events (e.g. atrial fibrillation), unstable or advanced ischemic heartdisease, hyperviscosity, or any other condition that the investigator believes islikely to interfere with evaluation of the study drug or with satisfactory conduct ofthe trial.
• Has a history of a malignant disease other than properly treated carcinoma in situ ofthe cervix or basal cell or squamous cell carcinoma of the skin within 24 months priorto enrolment.
• Has history of epilepsy or multiple episodes of migraine (defined as at least oneepisode within 6 months of enrolment) not completely controlled by medication.
• Is receiving steroids (oral or parenteral daily dose of ≥ 0.15 mg/kg/day of prednisoneor equivalent) OR other immunosuppressive drugs or chemotherapy.
• Females who are pregnant, breast feeding or planning a pregnancy during the course ofthe study. Women who become pregnant during the study will be withdrawn from thestudy.
• Has participated in another clinical study within 3 weeks prior to study enrolment.