Eculizumab costs at least US$440,000 for a single year of treatment and has been reported as one of the worlds most expensive drugs. Signs and symptoms
The classic sign of PNH is red discoloration of the urine due to the presence of hemoglobin  and hemosiderin from the breakdown of red blood cells. As the urine is more concentrated in the morning, this is when the color is most pronounced. This phenomenon mainly occurs in those who have the primary form of PNH, who will notice this at some point in their disease course. The remainder mainly experience the symptoms of anemia, such as tiredness, shortness of breath, and palpitations.A small proportion of patients report attacks of abdominal pain, difficulty swallowing and pain during swallowing, as well as erectile dysfunction in men; this occurs mainly when the breakdown of red blood cells is rapid, and is attributable to spasm of smooth muscle due to depletion of nitric oxide by red cell breakdown products.Forty percent of people with PNH develop thrombosis (a blood clot) at some point in their illness. This is the main cause of severe complications and death in PNH.
Necrobiotic xanthogranuloma (also known as "necrobiotic xanthogranuloma with paraproteinemia") is a multisystem disease that affects older adults, and is characterized by prominent skin findings. : 707

See also
List of cutaneous conditions

References


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