Autoimmune Hemolytic Anemia
Conditions
Interventions
C1 inhibitor (Cinryze)
Sponsors
Academic Medical Center (AMC)
Eligibility
Inclusion criteria
Inclusion criteria: • Positive (=1+) monospecific antiglobulin test for C3b and/or C3d with/without positivity for IgM OR strongly positive (=3+) monospecific antiglobulin test for C3b and/or C3d with positivity for IgG • Indication for a transfusion with at least 2 red packed cell concentrates based on the clinical assessment by the hematologist in charge • Hemoglobin value at least < 5 mmol/L (8 g/dl) with/without clinical symptoms • Clinical signs of hemolysis: not-detectable haptoglobin (mandatory) and increased lactate dehydrogenase (LDH) eventually combined with hyperbilirubinemia (increased direct and/or indirect bilirubin), lactate. • Age = 18 years • Written informed consent • Women of child bearing potential must have had a negative serum pregnancy test 7 days prior to the start of study drug
Exclusion criteria
Exclusion criteria: • History of arterial and/or venous thromboembolic events in the absence of an actual treatment with Vitamin K-antagonists • Concomitant use of therapeutic doses of heparin • Female patients who are pregnant or breast feeding or adults of reproductive potential who are not using effective birth control methods. If barrier contraceptives are being used, these must be continued throughout the trial by both sexes. Oral contraceptives only are not acceptable. • Patients with known HIV seropositivity or chronic active hepatitis • Patients who have any severe and/or uncontrolled medical condition or other conditions that could affect their participation in the study such as: – cerebrovascular accidents = 6 months before study drug start – uncontrolled hypertension
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| •improvement of recovery of RBC transfusion, •inhibition of complement activation and deposition on RBC via the classical pathway of complement, •safety | — |
Secondary
| Measure | Time frame |
|---|---|
| • Attenuates the pro-inflammatory response in AIHA • Affects the response to the basic treatment targeting autoantibody production | — |
Contacts
Public ContactEdith van Dijkman
Amsterdam UMC, location AMC
Outcome results
None listed