A patient with Paroxysmal Noctural Hemoglobinuria (PNH) may develop a number of signs and symptoms associated with smooth muscle dystonia.
Mechanism: nitric oxide depletion during hemolytic episodes caused by (1) binding with free plasma hemoglobin and (2) release of erythrocyte arginase which reduces nitric oxide synthesis.
Signs and symptoms may include:
(1) abdominal pain
(2) esophageal spasms
(3) erectile dysfunction
(4) fatigue
Risk factors:
(1) percent of granulocytes marking as PNH > 60%
(2) hemolytic episode with hemoglobinuria
(3) low serum haptoglobin levels
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