FIELD: medicine.
SUBSTANCE: invention relates to medicine, specifically to immunology, and can be used to administer a MASP-2 inhibitory agent that inhibits MASP-2-dependent complement activation for the manufacture of a medicament for treating a subject suffering from paroxysmal nocturnal hemoglobinuria (PNH), wherein the MASP-2 inhibitory agent comprises a monoclonal antibody against MASP-2 or an antigen-binding fragment thereof that specifically binds to a portion of SEQ ID NO: 6 and selectively inhibit MASP-2-dependent complement activation without substantially inhibiting the C1q-dependent complement pathway.
EFFECT: this allows to inhibit, prevent or reduce the severity of extravasal hemolysis in PNH patients undergoing treatment with a C5 inhibitor, such as eculizumab.
10 cl, 63 dwg, 15 tbl, 36 ex
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