Study to Evaluate Safety and Pharmacokinetics of BIVIGAM® in Primary Immune Deficiency Subjects Aged 2 to 16

Inclusion Criteria:

- Written informed consent/Assent

- Male or female between 2 and 16 years, inclusive, at time of Signing Informed
Consent/Assent

- Have a confirmed and documented clinical diagnosis of Primary Immune Deficiency
Disorder, including hypogammaglobulinemia or agammaglobulinemia.

- Have received IGIV therapy which was maintained at a steady dose (± 25% of the mean
dose) for at least 3 months prior to study entry, and have maintained a trough IgG
level at least 500mg/dL prior to receiving BIVIGAM®.

- Subjects and/or parents/legal guardians must be able to understand and adhere to the
study visit schedule and all other protocol requirements.

Exclusion Criteria:

- Known intolerance to immunoglobulins or comparable substances (e.g. vaccination
reaction).

- Known intolerance to proteins of human origin or known allergic reactions to
components of the study product(s).

- Any previous randomization/participation in this clinical study must be discussed with
and approved by the medical director (or designee).

- Inability or lacking motivation to participate in the study.

- Medical condition, laboratory finding, or physical exam finding (specify, e.g., vital
signs outside of specific range that precludes participation. Per lab results at the
Screening visit through Baseline.

- Confirmed Screening visit laboratory results ˃2.5 X ULN as defined for pediatric
populations for any of the following: ALT (alanine aminotransferase/SGPT), AST
(aspartate aminotransferase/SGOT), LDH (lactate dehydrogenase), BUN (blood urea
nitrogen), Serum creatinine

- Has selective IgA deficiency or demonstrated antibodies to IgA.

- History of thrombotic complications of IGIV therapy or history of (deep vein
thrombosis)DVT.

- Current use of daily corticosteroids (>10 mg of prednisone
equivalent/day),immunosuppressants or immunomodulators are not allowed unless approved
in advance by the medical monitor. Intermittent use of corticosteroids during the
study is allowed if medically necessary.

- Positive diagnosis of hepatitis B or hepatitis C.

- Positive human immunodeficiency virus (HIV) test.

- Subject has had a serious bacterial infection (SBI) within the last 3 months.

- Subject has an active infection and is receiving antibiotic therapy for the treatment
of this infection at the time of Screening. Note: if the subject is deemed a Screen
Failure due to a nonserious active infection requiring antibiotic therapy, the subject
may be rescreened 3 or 4 weeks (depending on drug administration schedule) after the
initial screening.

- Subject has a history of thrombotic events (including deep vein thrombosis, myocardial
infarction, cerebrovascular accident and pulmonary embolism) within 6 months before
1st IGIV dose or has preexisting risk factors for thrombotic events.

- Acquired medical condition known to cause secondary immune deficiency such as chronic
lymphacitic leukemia, lymphoma or multiple lymphoma.

- Subjects with protein-losing enteropathies, hypoalbuminaemia.

- Females taking oral contraceptives.

- Pregnancy or unreliable contraceptive measures or lactation period (females of
childbearing potential (female capable of becoming pregnant) only. Males capable of
reproduction must agree to a double barrier method of contraception during their study
participation.