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Thrombin generation in children with sickle cell disease: relationship with age, hemolysis, transcranial d oppler velocity, and hydroxyurea treatment
Author(s) -
Noubouossie Denis C. F.,
Lê Phu Q.,
Rozen Laurence,
Ziereisen France,
Willems Dominique,
Demulder Anne,
Ferster Alina
Publication year - 2013
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.12113
Subject(s) - hemolysis , transcranial doppler , medicine , gastroenterology , hemoglobin , thrombomodulin , thrombin , reticulocyte , platelet , endocrinology , immunology , biology , biochemistry , messenger rna , gene
Increased thrombin generation ( TG ) was described in sickle cell disease ( SCD ) children. The aim of this study was to characterize TG at the individual level and assess its relationship with age, hemolysis, transcranial Doppler velocity ( TCD ), and hydroxyurea treatment. Patients and methods TG was triggered in the platelet‐poor plasma using tissue factor and phospholipids with addition of thrombomodulin in 97 SCD at steady state and 80 control children. Patients and controls were aged from 2 to 20 years, and they were distributed in four categories of age: [2–5], [6–10], [11–15], and [16–20] years. For each subject, ratio of endogenous thrombin potential (r ETP ) and peak height (rPeak) was calculated as subject's value divided by the mean value of controls of the same age range. r ETP and rPeak of patients were considered abnormal when > mean + 2 SD of controls. LDH , total hemoglobin, and reticulocyte count were measured as markers of hemolysis. Data on hydroxyurea treatment and TCD were collected from medical records. Results Overall, 38.1% and 44.3% of patients showed elevated r ETP and rPeak, respectively. r ETP and rPeak decreased significantly with increasing age. In homozygous (SS) patients, TCD velocities and all markers of hemolysis correlated significantly with both r ET p and rPeak. Negative correlations were observed between these ratios and the duration of hydroxyurea treatment. Conclusion Elevated TG in SCD children is mainly related to younger age and to the intensity of hemolysis. There probably a link between TG and cerebral vasculopathy in these patients. Hydroxyurea may have a beneficial effect, which could be related to the duration of treatment.