Abstract
Background: Sickle cell disease is a major hereditary haemoglobinopathy in Basrah;Iraq. Stroke is one of the most devastating complications of Sickle cell disease and chronic erythrocytapheresis has become a cornerstone of its secondary prevention.
Objectives: To describe the clinical profile, complication burden and outcomes of Stroke in Sickle cell disease patients attending the Basrah Center for Hereditary Blood Diseases and to evaluate the safety and efficacy of erythrocytapheresis as a secondary Stroke-prevention strategy.
Methods: A descriptive study was conducted at Basrah Center for Hereditary Blood Diseases. Sixteen patients with confirmed Sickle cell disease and stroke who maintained on a regular erythrocytapheresis program.
Results: Twelve patients (75.0%) were male and four (25.0%) were female; ages ranged from 8 to 23 years (mean 15.1 years). A single prior Stroke was documented in 50% patients, two episodes in 18.75% and more than two episodes in 31.25%. Stroke recurrence showed 50.0% of children aged ≤10 years had ≥3 prior events.Cumulative transfusion exposure was variable (< 10 simple transfusions in 4/16, 10–30 in 5/16 and qualitatively reported as “frequent” in 6/16), and 4 of 16 patients had more than 25 cumulative erythrocytapheresis sessions. Service attitudes were 93.75% strongly agreed that the apheresis service is indispensable to the center, 93.75% were “very satisfied” with the service and 62.50% reported a marked improvement in life and function after enrolment.
Conclusions: Erythrocytopheresis combined with hydroxyurea provides effective and safe secondary Stroke prevention in Sickle cell disease patients at Basrah Center for Hereditary Blood Diseases, with a low recurrence rate (12.5%) and substantial improvements.