Table 1.

Clinical categories of patients with PNH clones

PNH categoryPNH granulocyte cloneClinical hemolysisLaboratory markers of hemolysisPNH symptomsRisk of thrombosisUtility 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 
PNH categoryPNH granulocyte cloneClinical hemolysisLaboratory markers of hemolysisPNH symptomsRisk of thrombosisUtility 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 
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