Therapy of Chronic Cold Agglutinin Disease With Eculizumab

Inclusion Criteria: * Individuals at least 18 years of age * Diagnosis of CAD defined by the combination of chronic hemolysis, cold agglutinin titer (IgM or IgA) \>64 at 4°C, positive direct antiglobulin test (DAT) when performed with polyspecific antiserum, negative (or only weakly positive) anti-IgG, and strongly positive with anti-C3d * LDH level \> 2 x upper limit of normal (ULN) * Clinical symptoms requiring treatment, such as anemia, anemia-related symptoms or hemolysis-related symptoms * Sufficient supplementation of iron, folic acid and vitamin B12 prior to treatment phase * Patient must be willing and able to give written informed consent; * Patients must be vaccinated against N. meningitidis at least 14 days prior treatment * Patient must avoid conception during the trial using a method that is most appropriate for their physical state and culture Exclusion Criteria: * Active aggressive lymphoma requiring therapy or an active non-lymphatic malignant disease other than basal cell carcinoma or CIS of the cervix * Liver disease with elevated LDH * Absolute neutrophil count \< 500/µL * Presence or suspicion of active bacterial or viral infection, in the opinion of the Investigator, at treatment start or severe recurrent bacterial infections * Participation in any other investigational drug trial or exposure to other investigational agent, device, or procedure within 30 days prior to screening period * Pregnant, breast-feeding, or intending to conceive during the course of the study, including the Post-treatment Phase * History of bone marrow/stem cell transplantation * Inability to cooperate or any condition that, in the opinion of the investigator, could increase the patient's risk by participating in the study or confound the outcome of the study * Hypersensitivity to eculizumab, to murine proteins or to one of the excipients