Paroxystic nocturnal hemoglobinuria, also called HPN, is a rare disease of genetic origin, characterized by changes in the membrane of red blood cells, which lead to their destruction and elimination of red blood cell components in the urine, so it is considered chronic hemolytic.Anemia.
The term nocturnal refers to the time of day when the highest rate of destruction of red blood cells has been observed in people with the disease, but research has shown that hemolysis, the destruction of red blood cells, occurs at any time of the day in people.with hemoglobinuria.
- HPN has no cure.
- But treatment can be done through bone marrow transplantation and the use of Eculizumab.
- Which is the specific drug for the treatment of this disease.
- Learn more about Eculizumab.
The main symptoms of paroxystic nocturnal hemoglobinuria are
People with paroxystic nocturnal hemoglobinuria have an increased risk of thrombosis due to changes in the blood clotting process.
Diagnosis of paroxystic nocturnal hemoglobinuria is done through several tests, such as:
In addition to these tests, the hematologist may order complementary tests, such as the sucrose test and ham test, which aid in the diagnosis of paroxysm paroxystic hemoglobinuria, which is usually between the age of 40 and 50 and the person’s survival is around 10 to 15 years.
Treatment of nocturnal paroxystic hemoglobinuria can be performed with allogeneic hematopoietic stem cell transplantation and with the drug Eculizumab (Soliris) 300 mg every 15 days.This drug can be provided by the SUS by legal action.
Iron supplementation with folic acid is also recommended, as well as adequate nutritional and haematological follow-up.