Etiopathological mechanisms and clinical characteristics of hyperhemolysis syndrome in Spanish patients with thalassemia.

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; Aguinaco, Maria Reyes

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.

SPAIN; 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

Annals of Hematology, 2016, Vol 95, Issue 9, p1419

0939-5555

Academic Journal

10.1007/s00277-016-2733-8