Abstract

Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anemia (SCA), is hemoglobin disorders that increase the rate of morbidity and mortality. Aim of this study is to evaluate D- dimer level in patients with sickle cell anemia (SCA) under hydroxyurea (HU) treatment. A total of 90 subjects, Homozygous (SCD) patients (HbSS) treated with Hydroxyurea (HU) (n=30), homozygous (SCD) untreated with hydroxyurea (n=30) and healthy (Hb AA) controls (n=30) matched for age (4 - 20) years, gender and socioeconomic status were enrolled from sinnar pediatric hospital. Venous blood samples 1.8 ml were collected in Trisodium citrate, centrifuged at room temperature then plasma was separated. The effect of the SCD patient's treatment with hydroxyurea on D- dimer level was investigated using Snibe MAGLUMI fully-autochemiluminescence immunoassay (CLIA) analyzer. The results of D-dimer are significantly lower (0.93±0.52ng/ml) (p value 0.000) in (SCD) under (HU) and normal control (0.39±0.14) compared to (SCD) patients without (HU) treatment (4.61±1.76ng/ml). Also, according to clinical status, SCD under HU Treatment about 73% are steady, and crises which about 27 %, SCD without HU treatment steady about 20% and crises about 80%. This reflect the significant role of HU therapy in the beneficial clinical effect. In conclusion, our study revealed low D-dimer levels and improves clinical and hematological characteristic among (SCD) patients under hydroxyurea treatment comparing with (SCD) patient without hydroxyurea treatment, while age showed no effect on the D-dimer levels.

Highlights

  • Sickle cell disease is a chronic hemolytic disorder that is marked by tendency of hemoglobin molecules within red cells to polymerise and deform the red cell into a sickle shape resulting in characteristic vasoocclusive events and accelerated hemolysis [1,2]

  • The mean of D-dimer in study groups are (0.93±0.52ng/ml) in Sickle cell disease (SCD) with HU, (4.61±1.76) in SCD without HU that means the D- dimer level was significantly lower among SCD patients under HU treatment (p value 0.000) than SCD without HU as it described in table (1&2)

  • They have been assessed according to clinical status, SCD without HU treatment in crises are more than SCD under HU treatment which reflect the significant role of HU therapy in the beneficial clinical effect explained in Figure (I)

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Summary

Introduction

Sickle cell disease is a chronic hemolytic disorder that is marked by tendency of hemoglobin molecules within red cells to polymerise and deform the red cell into a sickle (or crescent) shape resulting in characteristic vasoocclusive events and accelerated hemolysis [1,2]. Studies on SCD patients at steady state patients from different geographic and demographic origins have shown elevated level of markers of coagulation activation [6,7] One of these marker is D-dimer (fibrin degradation product); a small protein fragment present in the blood after a blood clot is degraded by fibrinolysis. Fibrinolysis is the resulting of interactions among multiple plasminogen activators and inhibitors constituting the enzymatic cascade leading to the degradation of fibrin [8]. It is normally undetectable or detectable at a very low level unless the body is forming and breaking down blood clots as in sickle cell anemia patients.

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