Extension Study of Eculizumab in Patients With Transfusion Dependent Paroxysmal Nocturnal Hemoglobinuria (PNH)

An open-label extension study to evaluate long-term safety of eculizumab in PNH patients who
had completed the TRIUMPH (C04-001), SHEPHERD (C04-002), and X03-001 studies.

Inclusion Criteria:

- Patients who have fully completed the TRIUMPH, SHEPHERD, or X03-001 studies

- TRIUMPH patients who have discontinued receiving investigational drug prior to the
last visit of the study due to lack of efficacy or exacerbation of symptoms of PNH and
have completed all monthly safety and efficacy procedures

- Patient must be willing and able to give written informed consent

- Patient must avoid conception during the trial

Exclusion Criteria:

- Patients who have terminated early from the SHEPHERD or X03-001 studies

- Patients who have terminated early from the TRIUMPH study due to an adverse event

- Female who is pregnant, breast feeding, or intending to conceive during the course of
the study

- Any condition that could increase the patient's risk by participating in the study or
could confound the outcome of the study