Efficacy and Safety of Elizaria® vs. Soliris® in Patients With PNH

Inclusion Criteria:

1. Written informed consent for participation in the study.
2. Men and women aged 18 to 65 years at the time of signing the Informed Consent Form.
3. Established diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), confirmed by flowcytometry assessing the PNH red blood cell and white blood cell clone size, withintravascular hemolysis and current or previous concomitant clinical symptoms,irrespective of the need for blood transfusions and without signs of other disordersassociated with bone marrow failure.
4. For patients, who have not received Soliris® before inclusion in this study: lactatedehydrogenase (LDH) level 1.5 times the upper limit of normal or higher as assessed bythe central laboratory.
5. The patients receiving anticoagulants must take them at a stable dose for at least 4weeks prior to screening. Patients receiving warfarin must have a stable internationalnormalized ratio (INR) value. To confirm INR value stability, the patients have toprovide a blood INR test at least 4 weeks prior to screening. The second INRassessment will be performed at the screening visit.
6. Documented vaccination against meningococcal infections (Neisseria meningitidisserogroups A, C, Y and W-135) the protective immunity of which did not wear off, atleast 14 days prior to the administration of the first dose of the test or referencedrug and the patient's consent for revaccination against meningococcal infections (Neisseria meningitidis serogroups A, C, Y and W-135) during participation in thecurrent study if the protection from previous vaccination wears off.
7. Subjects' consent to use reliable contraceptive methods (the combination of at least 2methods, including barrier contraception, for example, condoms with spermicide) fromsigning the Informed Consent Form for up to 10 weeks after stopping therapy.

Exclusion Criteria:

1. Hypersensitivity to the test drug, reference drug and their components.
2. Hypersensitivity to the active substance or any other component of the vaccine used toprevent meningococcal infection or a lifethreatening reaction to a previouslyadministered vaccine containing similar ingredients.
3. Conditions associated with bone marrow failure and PNH clone (aplastic anemia,myelodysplastic syndrome, idiopathic myelofibrosis).
4. A history of infections caused by Neisseria meningitides.
5. Active systemic bacterial, viral, or fungal infection within 14 days prior to theadministration of the first dose of the test or reference drug.
6. Fever of 38°С or higher within 7 days prior to the administration of the first dose ofthe test or reference drug.
7. Hereditary complement deficiencies.
8. Patients planning to undergo or with a history of bone marrow transplantation.
9. Initial treatment cycle (induction phase) of Soliris®; completed treatment withSoliris® less than 70 days before study inclusion, not related to the current studyparticipation.
10. Vaccination with any live vaccine within 1 month prior to the administration of thefirst dose of the test or reference drug;
11. Concomitant diseases and conditions which may, in the Investigator's opinion,compromise the patient's safety in case of participation in the study or which couldaffect the safety data analysis in case of an exacerbation of this disease/conditionduring the study, including the following:

• Myocardial infarction or stroke within the last 3 months, severe arrhythmia, NYHAfunctional class III/IV heart failure;
• Psychiatric disorders;
• Immune and endocrine disorders which are not controlled with medications (including decompensated diabetes mellitus and thyroid disorders);
• Hematologic disorders requiring chemotherapy;
• Current or prior oncologic disorders, except for successfully treated basal cellcarcinoma;
• Decompensated liver diseases.

12. Acquired immunodeficiency syndrome or human immunodeficiency virus (HIV) infectionconfirmed by test results.
13. Active viral hepatitis B and/or C at screening with alanine aminotransferase (ALT)and/or aspartate aminotransferase (AST) levels 5 times the upper limit of normal orhigher.
14. Positive syphilis test results.
15. Body mass index (BMI) ≤17 kg/m2 or ≥30 kg/m2.
16. Pregnancy or breastfeeding.
17. History of tuberculosis, alcohol addiction, medication abuse, or drug addiction.
18. Patient's participation in any clinical studies and/or using of not approvedmedications in the Russian federation within 30 days before screening.