1. Etiopathological mechanisms and clinical characteristics of hyperhemolysis syndrome in Spanish patients with thalassemia.
- Author
-
Vagace, Jose, Cardesa, Rocío, Corbacho, Antonio, Vázquez, Teresa, de la Maya, Maria, Gonzalez, Fernando, Nieto, José, Urrutia, Emilia, Gómez, María, Pascual, Teresa, Aguinaco, Maria, Gervasini, Guillermo, Vagace, Jose Manuel, Cardesa, Rocío, Vázquez, Teresa, de la Maya, Maria Dolores, Gonzalez, Fernando Ataulfo, Nieto, José Bartolomé, Gómez, María Jesus, and Aguinaco, Maria Reyes
- Subjects
THALASSEMIA treatment ,HEMOLYSIS & hemolysins ,RETICULOCYTES ,BLOOD transfusion reaction ,IMMUNOHEMATOLOGY ,SPLENECTOMY ,PUBLIC health ,ANTIGENS ,APOPTOSIS ,BLOOD transfusion ,COMPLEMENT (Immunology) ,FLOW cytometry ,IMMUNITY ,SYNDROMES ,THALASSEMIA ,RETROSPECTIVE studies ,BETA-Thalassemia ,THERAPEUTICS - Abstract
Hyperhemolysis syndrome (HHS) is characterized by severe intravascular hemolysis with a decrease in the reticulocyte count, which is triggered and aggravated by transfusion and cannot be explained by standard immunohematological studies. A nationwide study was conducted in order to retrospectively identify thalassemia patients with HHS in Spain in order to assess pre-disposing mechanisms for this syndrome. For this, the expression of adhesion (CD49, CD36) and complement-related molecules (C3a, CD59) and the levels of reticulocyte apoptosis and macrophage activation were measured in 4 thalassemia patients with HHS, 14 patients without HHS, and 10 healthy subjects. Five of the six thalassemia patients had δβ-thalassemia. The patients were not alloimmunized prior to the syndrome, which was developed after the first transfusion in all but one case. Patients with δβ-thalassemia did not respond to corticoids or immunoglobulins; only splenectomy was successful. The expression of CD49 (α4β1 integrin) was far higher in patients who had experienced HHS (85.07 ± 18.46 vs. 46.28 ± 24.31; p < 0.01), and the difference remained significant after correcting by the number of molecules analyzed (Bonferroni p < 0.05). In our population, δβ-thalassemia was the most common hemoglobinopathy in patients with HHS. Furthermore, the risk to develop this syndrome may be associated with an increased expression of α4β1 integrin. [ABSTRACT FROM AUTHOR]
- Published
- 2016
- Full Text
- View/download PDF