title: Sickle cell anemia induces changes in peripheral lymphocytes E-NTPDase/E-ADA activities and cytokines secretion in patients under treatment.
abstract: Sickle cell anemia (SCA) is characterized by hemoglobin polymerization that results in sickle-shaped red blood cells. The vascular obstruction by sickle erythrocytes is often inflammatory, and purinergic system ecto-enzymes play an important role in modulating the inflammatory and immune response. This study aimed to evaluate the E-NTPDase and E-ADA activities in lymphocytes of SCA treated patients, as well as verify the cytokine profile in this population. Fifteen SCA treated patients and 30 health subjects (control group) were selected. The peripheral lymphocytes were isolated and E-NTPDase and E-ADA activities were determined. Serum was separated from clot formation for the cytokines quantification. E-NTPDase (ATP and ADP as substrate) and E-ADA (adenosine as substrate) activities were increased in lymphocytes from SCA patients (P<0.001). The TNF-α and IL-6 serum cytokines showed decreased on SCA patients comparing to control (P<0.001). The regulation of extracellular nucleotides released in response to hypoxia and inflammation through E-NTPDase and E-ADA enzymes represent an important control of purine-mediated in the SCA disease, avoiding elevated adenosine levels in the extracellular medium and consequent organ injuries in these patients. The pro-inflammatory cytokines decreased levels by use of hydroxyurea occur in attempt to reduce the pro-inflammatory response and prevent vaso-oclusive crisis.
authors.affiliation: Departamento de Microbiologia e Parasitologia, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil. Electronic address: liviagelain@gmail.com.
authors.forename: Lívia G
authors.initials: LG
authors.lastname: Castilhos
authors.affiliation: Departamento de Microbiologia e Parasitologia, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil.
authors.forename: Pedro H
authors.initials: PH
authors.lastname: Doleski
authors.affiliation: Departamento de Microbiologia e Parasitologia, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil.
authors.forename: Tatiana M D
authors.initials: TM
authors.lastname: Bertoldo
authors.affiliation: Departamento de Microbiologia e Parasitologia, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil.
authors.forename: Daniela F
authors.initials: DF
authors.lastname: Passos
authors.affiliation: Departamento de Microbiologia e Parasitologia, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil.
authors.forename: Claudia de M
authors.initials: Cde M
authors.lastname: Bertoncheli
authors.affiliation: Departamento de Microbiologia e Parasitologia, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil.
authors.forename: João F P
authors.initials: JF
authors.lastname: Rezer
authors.affiliation: Departamento de Microbiologia e Parasitologia, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil.
authors.forename: Josiane B
authors.initials: JB
authors.lastname: Schlemmer
authors.affiliation: Departamento de Microbiologia e Parasitologia, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, Centro de Ciências da Saúde, Universidade Federal de Santa Maria, Av. Roraima, 97105-900, Santa Maria-RS, Brazil. Electronic address: daniela.leal@ufsm.br.
authors.forename: Daniela B R
authors.initials: DB
authors.lastname: Leal
mesh_terms: D000243:Adenosine Deaminase / Q000097:blood*; D000251:Adenosine Triphosphatases / Q000097:blood*; D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000201:enzymology* / Q000628:therapy; D016022:Case-Control Studies; D016207:Cytokines / Q000097:blood*; D005260:Female; D006801:Humans; D008214:Lymphocytes / Q000201:enzymology*; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D016207:Cytokines; D000243:Adenosine Deaminase; D000251:Adenosine Triphosphatases; C027201:ectoATPase
keywords: Cytokines; E-ADA; E-NTPDase; Sickle cell anemia