ارتباط العمر و الجرعة و المدة بنجاعة المعالجة بالهيدروكسي يوريا لدى مرضى سوريين مصابين بداء الكريات المنجلية

الملخص EN

Sickle cell disease (SCD) is one of the oldest diagnosed diseases on the molecular level.

However, medication for SCD is limited only for hydroxyurea.

The efficacy of hydroxyurea is mainly due to its capacity to raise fetal hemoglobin (HbF) levels, which in turn inhibit sickling process and decrease the frequency of painful crises.

Multiple factors may affect the treatment efficacy of hydroxyurea like dose, treatment duration and age.

In Syria, no study has screened the efficacy of hydroxyurea before.

Our study included 76 patients diagnosed with sickle cell disease, 44 of them had been treated with hydroxyurea.

Hematological investigations included: complete blood count (CBC) and HbF quantification by high performance liquid chromatography (HPLC) method.

Changes in HbF levels for 24 patients, who had available recorded baseline at initiation of hydroxyurea has been calculated (Δ HbF).

HbF levels were significantly higher in patients taking hydroxyurea than patients who were not under treatment.

For the treated group, patients who had started treatment at age younger than five years old have, with significant difference, higher hydroxyurea induced HbF (HU–HbF) levels than those who had started treatment at older age, without any significant difference in (Δ HbF) between the two groups.

However, no significant difference was noticed in (HU–HbF) levels, according to dose variation (less or more than 15 mg/kg/day) or treatment duration (less than 2 year or 2 years and more) This study provide the first evidence for hydroxyurea efficacy in the used dosage in sickle cell disease patients in Syria, with having a better response, when hydroxyurea treatment initiated before the age of five years.