STAFF BOARD
Article
CHAIRMAN
Virginia Hernández Corredoira
EDITOR IN CHIEF
Manuela Velázquez Prieto
Jaime E. Poquet Jornet
MARKETING MANAGER
EDITORIAL BOARD
Ramón Jódar Masanés
Lluís Campins Bernadas
Tomás Casasín Edo
Juan Carlos Juárez Giménez
Carles Quiñones Ribas
Volume 21 - Issue 5, September-October 2019
SHORT REPORTS
EIGHT YEARS OF EXPERIENCE WITH ECULIZUMAB IN A CASE OF PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
GONZÁLEZ PÉREZ YARED, NUÑEZ RODRÍGUEZ JESICA, APIÑANIZ APIÑANIZ REBECA, NEBOT VILLACAMPA MARIA JOSÉ, CALVO ARAGÜETE MARÍA EUGENIA, CASO GONZÁLEZ ALICIA, MARIN GORRICHO RAQUEL, CASAJUS NAVASAL ANDREA


Paroxysmal nocturnal hemoglobinuria is a rare clonal disease of hematopoietic stem cells. These hematopoietic cells present a deficit of surface membrane anchorage proteins such as DAF (Complement Degradation Accelerator Factor, CD55) and MIRL (Membrane Reactive Lysis Inhibitor, CD59). This total or partial absence produces a greater susceptibility of erythrocytes to complement-mediated hemolysis. Eculizumab is a monoclonal antibody indicated in the paroxysmal nocturnal hemoglobinuria, capable of blocking the complement protein C5 avoiding its activation and, therefore, hemolysis. We present the case of a woman diagnosed with paroxysmal nocturnal hemoglobinuria resistant to the use of systemic corticosteroids with a poor quality of life, an important amount of symptoms and hospitalizations in several occasions. After eight years in treatment with eculizumab the patient presents a good drug tolerance, a better quality of life and a decrease in the value of lactate dehydrogenase, stabilization of the value of hemoglobin and a reduction on the number of units of packed red cells transfused.

ECULIZUMAB – PAROXYSMAL NOCTURNAL HEMOGLOBINURIA – RARE DISEASES



Fill out the form below to buy this article

Name and surname:

DNI/NIF ID/VAT IDENTIFICATION No.:

Contact Email:

Phone:

Address:

Zip code:

City:

Province:

Country:

*I have read and accept the privacy policy
Price : 30,25