Secondary Immune Deficiency Disorder
Conditions
Keywords
chronic lymphocytic leukemia
Brief summary
Patients with chronic lymphocytic leukemia (CLL) are at increased risk of infections as compared to age matched controls, with infections being a major cause of morbidity and mortality. Previous studies have shown that patients with CLL have both hypogammaglobinemia and impaired humoral immunity as defined by vaccine responses to both polysaccharide and peptide antigens. Attempts at decreasing infections in CLL have included therapy with prophylactic antibiotics and intravenous immunoglobulin. In general clinical practice and in previous studies, patients have started IV immunoglobulin replacement therapy if they have a history of serious infection or hypogammaglobinemia (defined as Immunoglobulin G below 500-600 g/dL), but vaccine responses have not been evaluated. This study will identify CLL patients with humoral immunodeficiency by checking both Ig levels and vaccines responses. In patients with impaired humoral immunity, the investigators will use subcutaneous immunoglobulin replacement to show this intervention will increase Ig levels, protective antibody titers, and be well tolerated.
Interventions
DRUGHizentra
Subjects will receive Hizentra 0.4 mg/kg qweekly subcutaneously for 6 months (24 weeks)
Sponsors
Rochester General Hospital
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Diagnosis of chronic lymphocytic leukemia * Medically stable, with expected survival of \> 1 year * Able to understand and willingness to sign a written informed consent * Able to comply with study procedures
Exclusion criteria
* Previously diagnosed primary immunodeficiency * Additional immunosuppressive states as assessed by the primary or co investigators * Ongoing therapy with Ig replacement * Serum IgG \< 500 mg/dL
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Identify patients with CLL with humoral immunodeficiency despite serum IgG > 500 mg/dL. | 10 months | Incidence of humoral immunodeficiency |
| Number of patients who increase IgG to above 700 mg/dL | 10 months | Effectiveness of Hizentra to change IgG levels |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Number of patients who increase diphtheria IgG to above 0.1 IU/mL, tetanus IgG to above 0.1 IU/mL, and IgG to streptococcus pneumonia to above 1.3 mcg/mL | 10 months | Effectiveness of Hizentra to change specific antibody titers in CLL |
| Number of subjects with treatment-related adverse effects as assessed by CTCAE v4.0 | 10 months | Safety and tolerability of Hizentra in CLL |
| Quality of life (Short Form 36) on subcutaneous Ig replacement therapy a 0-100 scale, with lower scores indicating higher disability/lower quality of life | 10 months | Does Hizentra change quality of life scores in patients with CLL |
| Track the number of infections requiring antibiotics, further characterized per severity as defined in previous studies | 10 months | Does Hizentra change the rate of non-neutropenic infections in CLL |
Countries
United States
Outcome results
None listed