Paroxysmal nocturnal hemoglobinura (PNH) is an acquired rare disorder characterized the complement system destructing red blood cells to early. Red blood cells are missing the PIG-A gene that allows glycosyl-phosphatidylinositol (GPI) to help certain proteins stick to cells. PNH is the only hemolytic anemia caused by an acquired (rather than inherited) intrinsic defect in the cell membrane.
The worldwide prevalence is estimated in the range of 1–5 cases per million.
Complete blood count- Red and white blood cell counts and platelet counts may be low.
Flow cytometry- checks for accelerating factors of the red and white blood cells.
Coombs test- detects antibodies against red blood count.
Haptoglobin test- measures haptoblobin to see the rate of red blood cell death.
Monitor flow cytometry every 6 months
Soliris (eculizumab)- Soliris is indicated for the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis.