Human Immune Globulin in Treating Patients With Primary Amyloidosis That is Causing Heart Dysfunction

OBJECTIVES:

• Establish the maximum tolerated dose of human immune globulin intravenous (IGIV) given weekly for the first 3 months and then bi-weekly for 9 additional months in patients with cardiac-associated primary light chain-associated (AL) amyloidosis.
• Determine the safety, pharmokinetics, and therapeutic efficacy as evidenced by titers of serum fibril-reactive immunoglobulin G (IgG) antibodies pre- and post-IGIV infusions.
• Demonstrate stable or improved organ function.

OUTLINE: Patients receive human immune globulin IV (IGIV) once weekly for 3 months and then once biweekly for 9 months, for a total of 12 months in the absence of disease progression or unacceptable toxicity.

Patients undergo blood sample collection to measure serum anti-fibril antibody titers pre- and post- IGIV infusion for assessing safety and response to treatment.

Inclusion criteria

• Confirmed diagnosis of cardiac-associated primary (AL) amyloidosis based on accepted clinical and laboratory criteria
• Patients must have heart involvement as evidenced by elevated serum brain natriuretic peptide (BNP), troponin levels, and/or 2D echocardiography evidence of a thickened intraventricular septum (IVS).
• Life expectancy > 3 months
• Prior or concurrent chemotherapy or other drug-based anti-AL regimes allowed

Exclusion criteria

• Non-AL amyloidosis
• New York Heart Association (NYH) class IV heart disease
• Significant comorbidity (e.g., uncontrolled infection, diabetes, or other serious illnesses)