Table 2.

Clinical indications for PNH testing

Intravascular hemolysis as evidence by hemoglobinuria or elevated plasma hemoglobin.

Evidence of unexplained hemolysis with accompanying:

Iron‐deficiency, OR

Abdominal pain or esophageal spasm, OR

Thrombosis, OR

Granulocytopenia and/or thrombocytopenia

Other acquired Coombs‐negative, nonschistocytic, noninfectious hemolytic anemia

Thrombosis with unusual features:

Unusual sites

Hepatic veins (Budd‐Chiari Syndrome)

Other intra‐abdominal veins (portal, splenic, splanhnic)

Cerebral sinuses

Dermal veins

With signs of accompanying hemolytic anemia

With unexplained cytopenia

Evidence of bone marrow failure:

Suspected or proven aplastic or hypoplastic anemia

Refractory cytopenia with unilineage dysplasia

Other cytopenias of unknown etiology after adequate workup

Borowitz et al.9