Fourth day to the knowledge of the night Paroxistica Hemoglobinuria (HPN) held in Barcelonas Hospital Clínic.

Barcelona, July 2012.- diagnosis of a patient with paroxysmal Paroxistica night (HPN) may take up to 10 years, a fact that causes a delay in the treatment of disease, as they have highlighted the specialists gathered in the sixth day to the knowledge of the HPN ” held at the Hospital Clínic of Barcelona.

Paroxysmal nocturnal hemoglobinuria (PNH) is a ultra-rara disease that affects about 250 people in Spain and that is characterized by the destruction of red blood cells (hemolysis) and can cause thrombosis, main cause of death among these patients.

It’s one of the few ultra-raras diseases with treatment showing same rates of survival than the general population. Hence, according to experts, it is so important to properly diagnose this type of disease so rare and subsequently manage the treatment.

Flow cytometry is one of the methods to diagnose the HPN, but it can take years to request the test, so explained Dr. Neus Villamor, Senior Consultant of the Centre for biomedical diagnosis of the Hospital Clínic of Barcelona.

The Paroxistica nocturnal Hemoglobinuria is ultra-raras few diseases with a treatment that allows the patient to have the same rates of survival than the general population. Eculizumab, drug approved by the FDA (Food and Drug Administration) and EMA (European Medicines Agency) in 2007, is the only one that has shown to improve the quality of life of HPN patients and avoid any risk of thrombosis, main cause of death of this disease.

The meeting also attended the Anna Gaya doctors and Alvaro urban, the service of Hematology and head of the Institute of diseases Hemato-oncological del Marín Clinic of Barcelona, respectively. Immaculate Gómez, a patient of HPN, has also told in first-person experience with the disease.

To conclude the Conference, Jordi Cruz, President of the patients Association HPN, organizer of the event, spoke to attendees of the importance of associative in cases of rare diseases, since the patient may have more and better access to information, psychological support or collaborate to continue investigating.

paroxysmal Paroxistica night

paroxysmal nocturnal hemoglobinuria (PNH) is a ultra-rara disease that affects about 250 patients in Spain. It is a genetic disorder in which normal natural proteins do not adhere properly to the red blood cells and, consequently, they are unprotected from the immune system and are destroying prematurely and cronica1. It is a ultra-rara disease that progressively impairs patients and constitutes a real life threat.