Table 1.

Signs and symptoms of PNH

Subjective 
Fatigue, lethargy, asthenia, loss of sense of well-being 
Abdominal pain 
Dyspnea 
Chest pain 
Odynophagia* 
Male impotence 
Headache 
Objective 
Hemoglobinuria 
Scleral icterus, jaundice 
Thromboembolic events 
Laboratory abnormalities 
Cytopenias (anemia, thrombocytopenia, leukopenia, pancytopenia) 
Evidence of intravascular hemolysis (elevated LDH, elevated indirect bilirubin, low haptoglobin) 
Flow cytometry demonstrates deficiency of GPI-AP on a portion of red cells, granulocyte, and monocytes 
Iron deficiency 
Hemosiderinuria 
Subjective 
Fatigue, lethargy, asthenia, loss of sense of well-being 
Abdominal pain 
Dyspnea 
Chest pain 
Odynophagia* 
Male impotence 
Headache 
Objective 
Hemoglobinuria 
Scleral icterus, jaundice 
Thromboembolic events 
Laboratory abnormalities 
Cytopenias (anemia, thrombocytopenia, leukopenia, pancytopenia) 
Evidence of intravascular hemolysis (elevated LDH, elevated indirect bilirubin, low haptoglobin) 
Flow cytometry demonstrates deficiency of GPI-AP on a portion of red cells, granulocyte, and monocytes 
Iron deficiency 
Hemosiderinuria 
*

May be a consequence of sequestration of nitric oxide by plasma-free hemoglobin.

A presenting symptom in approximately 25% of cases.

May involve an unusual site (skin, splanchnic veins [Budd-Chiari syndrome], cerebral veins).

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