Table 1.
PNH category | PNH granulocyte clone | Clinical hemolysis | Laboratory markers of hemolysis | PNH symptoms | Risk of thrombosis | Utility of complement inhibitor therapy |
---|---|---|---|---|---|---|
Bone marrow failure with an associated PNH clone | ||||||
Subclinical (majority of patients) | Small (usually <30%) | Absent | May be present, depending on clone size | Absent | Low | Do not benefit from complement inhibitor |
Symptomatic | Moderate | May be present | May be present | May be present | Intermediate | Some patients may benefit from complement inhibitor |
Classical PNH | Large (usually >50%) | Present | Present | Present | High | Complement inhibitor improves outcomes |